PEMAHAMAN KEBERAGAMAAN DAN GERAKAN KELOMPOK FPI SURABAYA

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Syamsul Ma’arif

2017-08-01

  Sikap-sikap FPI (Front Pembela Islam di Indonesia selama ini terkesan ‘frontal’ dan melawan setiap bentuk kemunkaran. Aksi mereka, sebenarnya bisa dikatakan sebagai salah satu bentuk ekspresi terdalam dari sekelompok orang muslim dalam mengartikulasikan ajaran-ajaran agama yang dipeluknya, atau sebagai bentuk sikap “kesalehan” dalam memandang setiap keyakinan yang berbeda dengan keyakinan yang diyakininya. Apalagi dalam memandang persoalan yang jelas-jelas dianggap sebagai bentuk kemaksiatan, kesesatan dan penodaan agama. Maka respons dan reaksi mereka, sebagaimana hasil penelitian yang telah dilakukan penulis di Surabaya adalah dalam rangka menegakkan prinsip keimanan yang dianggap “benar” tadi. Tulisan ini bertujuan untuk memberikan informasi yang komprehensif tentang faktor apa saja yang melatari lahirnya kelompok-kelompok keagamaan kontemporer—terutama sekali FPI di Surabaya. Sekaligus mengetahui pemahaman keagamaan, aktifitas dan gerakan-gerakan kelompok FPI di daerah tersebut. Gambaran hasil penelitian ini pada akhirnya diharapkan mampu mengeliminasi kemungkinan kesalahpahaman informasi yang menjadi sebab timbulnya prejudice, dan stereotype yang merupakan langkah awal terjadinya konflik atau mengatasi konflik antar komunitas agama secara komprehensif. Sebab, dengan diketahui keanekaragaman kelompok-kelompok keagamaan dengan dinamika gerakannya  akan lebih memudahkan masyarakat agama untuk saling belajar satu sama lain.

Comparison of Risk Factors in Necrotizing Enterocolitis among Infants in Neonatal Intensive Care Unit

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Ferda Ozlu

2013-08-01

Full Text Available Purpose: Necrotizing enterocolitis is one of the important problems of premature infants. The incidence is about 1-5% in infants followed in neonatal care units and inversely related to gestational age and birth weight. Materials and Methods: In this study, 31 infants with necrotizing enterocolitis and 31 infants with similar gestational age and birth weight as control group hospitalized in Cukurova University Neonatal Care Unit between 1 January 2001-31 January 2004 were evaluated. Results: The incidence of necrotizing enterocolitis in this period was 1.4 (31/2214 admission. Mean gestational age was 30.5  3.2 weeks (25-36, mean birth weight was 1331  384 (730-2150 grams while 71% was younger than 32 gestational weeks and 67.7% was under 1500 grams. The signs of NEC were detected at a mean of 11.2  10. (2-38 days. Twenty-six (83.9% were being fed at the time of the necrotizing enterocolitis signs appeared. According to the Walsh and Kliegman classification, 19 (61.3% infants were in stage 1 (17 were 1a, 2 were 1b; 3 (9.6% infants were in stage 2a, 9 (%29.1 infants were in stage 3 (7 were in 3b. Blood culture was positive in 7 (%22.6 infants with predominance of gram negative microorganisms (5 infants. Eleven (%35.5 infants were exitus, 12 were discharged. Hypoxia, respiratory distress syndrome, intraventricular hemorrhage and umbilical catheterization were significant risk factors in necrotizing enterocolitis . Thrombocytopenia, leucopenia and high C-reactive protein levels were significantly high in necrotizing enterocolitis group. Breast feeding is significantly high in control group. Conclusion: Necrotizing enterocolitis, is a leading cause of morbidity and mortality in neonatal intensive care units. Early breast feeding with small amounts, increasing amount of milk slowly, antenatal steroids, caring hygiene rules can prevent the development of it. [Cukurova Med J 2013; 38(4.000: 642-647

Necrotizing enterocolitis: current perspectives

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Yajamanyam PK

2014-03-01

Full Text Available Phani Kiran Yajamanyam,1 Shree Vishna Rasiah,1 Andrew K Ewer1,2 1Neonatal Unit, Birmingham Women's Hospital NHS Foundation Trust, 2School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, UK Abstract: Necrotizing enterocolitis is the most common gastrointestinal emergency in neonates, particularly in those born very preterm. It is a leading cause of morbidity and mortality, especially in extremely low birth weight infants. Despite extensive research, the pathophysiology of necrotizing enterocolitis remains unclear and therapeutic options are limited. Multiple risk factors have been reported, but most are associated with prematurity and its complications. This makes management very challenging in vulnerable preterm infants. In this review, we focus on the risk factors and some of the current research in this area, particularly studies aimed at early detection and potential preventive measures for this potentially lethal condition. Keywords: necrotizing enterocolitis, preterm infants, prematurity, probiotics

Non-IgE-mediated gastrointestinal food allergies in children.

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Caubet, Jean-Christoph; Szajewska, Hania; Shamir, Raanan; Nowak-Węgrzyn, Anna

2017-02-01

Non-IgE-mediated gastrointestinal food allergic disorders (non-IgE-GI-FA) including food protein-induced enterocolitis syndrome (FPIES), food protein-induced enteropathy (FPE), and food protein-induced allergic proctocolitis (FPIAP) are relatively uncommon in infants and young children, but are likely under-diagnosed. Non-IgE-GI-FA have a favorable prognosis, with majority resolving by age 3-5 years. Diagnosis relies on the recognition of symptoms pattern in FPIAP and FPIES and biopsy in FPE. Further studies are needed for a better understanding of the pathomechanism, which will lead eventually to the development of diagnostic tests and treatments. Limited evidence supports the role of food allergens in subsets of constipation, gastroesophageal reflux disease, irritable bowel syndrome, and colic. The immunologic pathomechanism is not fully understood and empiric prolonged avoidance of food allergens should be limited to minimize nutrient deficiency and feeding disorders/food aversions in infants. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Colonic stenosis post-necrotizing enterocolitis in term newborn with acquired cytomegalovirus infection.

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Marseglia, L; Manti, S; D'Angelo, G; Lima, M; Impellizzeri, P; Romeo, C; Gitto, E

2015-01-01

Necrotizing enterocolitis is a gastrointestinal emergency typical of premature infants. Intestinal strictures infrequently complicate medical or surgical treatment of necrotizing enterocolitis. Postnatal cytomegalovirus infection with gastrointestinal linvolvement has occasionally been described in subjects with necrotizing enterocolitis. We report the case of a full term infant presenting necrotizing enterocolitis, acquired cytomegalovirus infection and post necrotizing enterocolitis colonic stricture.List of abbreviations: necrotizing enterocolitis = NEC,cytomegalovirus = CMV. Celsius.

Probiotics in premature infants: focus on necrotising enterocolitis

African Journals Online (AJOL)

Necrotising enterocolitis (NEC) is predominantly seen in premature infants and is the leading .... Because neonates are often intolerant to large enteral volumes, the ... of necrotizing enterocolitis in very low birth weight infant. Pediatrics. 2005 ...

Surgical management of radiation enterocolitis

International Nuclear Information System (INIS)

Sakaguchi, Masahiro; Katsumi, Masaharu; Ishimoto, Kiwao; Yamamoto, Shinji; Yukawa, Hirofumi; Koh, Kenzo; Yamaguchi, Kazuya; Ohta, Masataka; Hayashido, Motonori

1986-01-01

Radiotherapy for malignant tumors is effective and established. However, there are many complications in radiotherapy. A typical complication is radiation enterocolitis. It is difficult to treat severe histological damage to the irradiated tissues. This paper reports our experience with 26 patients with radiation enterocolitis for whom surgical treatment was given during a period of 16 years in our surgical department. The most frequent original disease requiring irradiation was cancer of the cervix uteri (15 cases) which was followed by cancer of the urinary bladder (7 cases). All of these patients had received external 60 Co irradiation. The ileum was the most frequent site of the lesion, followed by the rectum, sigmoid and descending colon. The period between the end of irradiation and the onset of enterocolitis ranged from 2 months to 10 years, with an average of 28 months. The main symptoms were intestinal obstruction in the ileum, and melena and fistula in the rectum and sigmoid colon. Intestinal resection was performed in 10 patients, a bypass operation in 4, and colostomy in 12. As to types of intestinal suture in these cases, Gambee's single layer method was thought useful from our experience. Colostomy as surgical management of melena and fistula did not yield satisfactory results. (author)

The use of geological data from pilot holes for predicting FPI (full perimeter intersection) fractures

International Nuclear Information System (INIS)

Joutsen, A.

2012-02-01

Posiva Oy is responsible for preparation of final disposal of spent nuclear fuel in Olkiluoto. The knowledge about existing network of fractures is important for the safety and feasibility of the final repository. The bedrock properties essential for safety case are analyzed in investigations of Rock Suitability Criteria (RSC). One subtask in RSC is avoidance of large (long) fractures adjacent to disposal holes. The long fractures have been defined in tunnel mapping to indicate tunnel cross-cutting fractures (TCF) or full perimeter intersections (FPI). The purpose for this study was to evaluate the possibility of recognizing FPIs from drill cores by their geological properties. The study was carried out by correlating FPIs mapped from the ONKALO tunnel to the pilot holes logging data with a view of finding out which fracture in the pilot hole corresponds to the FPI in the tunnel. It was also estimated what kind of geological properties does FPIs commonly have in the tunnel and how does these properties differ from the FPI correlated pilot hole fractures. The data sources for this study are the pilot holes from ONK-PH8 to ONK-PH14 and the systematic geological mapping data. The FPIs used in this study usually follow the general trends of the fracturing in the Olkiluoto bedrock. The fracture surface profiles are principally undulating and a striation can be often seen on the fracture surfaces. The FPIs are frequently moderately to intensely altered with diverse filling mineralogy and thick fracture fillings in comparison to the regular fractures. The FPI correlated pilot hole fractures have slightly different properties in comparison to the FPIs. These fractures have wider range of different fracture surface profiles and are slightly less altered than the FPIs. Filling mineralogy follows the trends of the FPIs but filling thicknesses are thinner. These differences probably occur because of the variable uncertainties related to the correlation and to the fact that the

The use of geological data from pilot holes for predicting FPI (full perimeter intersection) fractures

Energy Technology Data Exchange (ETDEWEB)

Joutsen, A.

2012-02-15

Posiva Oy is responsible for preparation of final disposal of spent nuclear fuel in Olkiluoto. The knowledge about existing network of fractures is important for the safety and feasibility of the final repository. The bedrock properties essential for safety case are analyzed in investigations of Rock Suitability Criteria (RSC). One subtask in RSC is avoidance of large (long) fractures adjacent to disposal holes. The long fractures have been defined in tunnel mapping to indicate tunnel cross-cutting fractures (TCF) or full perimeter intersections (FPI). The purpose for this study was to evaluate the possibility of recognizing FPIs from drill cores by their geological properties. The study was carried out by correlating FPIs mapped from the ONKALO tunnel to the pilot holes logging data with a view of finding out which fracture in the pilot hole corresponds to the FPI in the tunnel. It was also estimated what kind of geological properties does FPIs commonly have in the tunnel and how does these properties differ from the FPI correlated pilot hole fractures. The data sources for this study are the pilot holes from ONK-PH8 to ONK-PH14 and the systematic geological mapping data. The FPIs used in this study usually follow the general trends of the fracturing in the Olkiluoto bedrock. The fracture surface profiles are principally undulating and a striation can be often seen on the fracture surfaces. The FPIs are frequently moderately to intensely altered with diverse filling mineralogy and thick fracture fillings in comparison to the regular fractures. The FPI correlated pilot hole fractures have slightly different properties in comparison to the FPIs. These fractures have wider range of different fracture surface profiles and are slightly less altered than the FPIs. Filling mineralogy follows the trends of the FPIs but filling thicknesses are thinner. These differences probably occur because of the variable uncertainties related to the correlation and to the fact that the

Radiosynthesis of a new PSMA targeting ligand ([18F]FPy-DUPA-Pep)

International Nuclear Information System (INIS)

Malik, Noeen; Machulla, Hans-Juergen; Solbach, Christoph; Winter, Gordon; Reske, Sven N.; Zlatopolskiy, Boris

2011-01-01

Due to the specificity of expression of PSMA (prostate specific membrane antigen) particularly in prostate cancer cells (e.g. LNCaP), numerous PSMA ligands have been synthesized until now. In the current study, we synthesized DUPA-Pep having 2-[3-(1,3-dicarboxypropyl)ureido]pentanedioic acid (DUPA) linked via 8-aminooctanoic acid to two phenylalanine residues and chose 6-[ 18 F]fluoronicotinic acid 2,3,5,6-tetrafluorophenyl ester [ 18 F]FPy-TFP as a prosthetic group for coupling. [ 18 F]FPy-DUPA-Pep was obtained in a radiochemical yield of 48±0.9% (decay uncorrected) within 50 min with a chemical purity of >98%.

Radiosynthesis of a new PSMA targeting ligand ([18F]FPy-DUPA-Pep).

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Malik, Noeen; Machulla, Hans-Jürgen; Solbach, Christoph; Winter, Gordon; Reske, Sven N; Zlatopolskiy, Boris

2011-07-01

Due to the specificity of expression of PSMA (prostate specific membrane antigen) particularly in prostate cancer cells (e.g. LNCaP), numerous PSMA ligands have been synthesized until now. In the current study, we synthesized DUPA-Pep having 2-[3-(1,3-dicarboxypropyl)ureido]pentanedioic acid (DUPA) linked via 8-aminooctanoic acid to two phenylalanine residues and chose 6-[(18)F]fluoronicotinic acid 2,3,5,6-tetrafluorophenyl ester [(18)F]FPy-TFP as a prosthetic group for coupling. [(18)F]FPy-DUPA-Pep was obtained in a radiochemical yield of 48±0.9% (decay uncorrected) within 50 min with a chemical purity of >98%. Copyright © 2011 Elsevier Ltd. All rights reserved.

Radiosynthesis of a new PSMA targeting ligand ([{sup 18}F]FPy-DUPA-Pep)

Energy Technology Data Exchange (ETDEWEB)

Malik, Noeen, E-mail: noeen.malik@uniklinik-ulm.d [Clinic for Nuclear Medicine, University Hospital, Ulm (Germany); Machulla, Hans-Juergen; Solbach, Christoph; Winter, Gordon; Reske, Sven N.; Zlatopolskiy, Boris [Clinic for Nuclear Medicine, University Hospital, Ulm (Germany)

2011-07-15

Due to the specificity of expression of PSMA (prostate specific membrane antigen) particularly in prostate cancer cells (e.g. LNCaP), numerous PSMA ligands have been synthesized until now. In the current study, we synthesized DUPA-Pep having 2-[3-(1,3-dicarboxypropyl)ureido]pentanedioic acid (DUPA) linked via 8-aminooctanoic acid to two phenylalanine residues and chose 6-[{sup 18}F]fluoronicotinic acid 2,3,5,6-tetrafluorophenyl ester [{sup 18}F]FPy-TFP as a prosthetic group for coupling. [{sup 18}F]FPy-DUPA-Pep was obtained in a radiochemical yield of 48{+-}0.9% (decay uncorrected) within 50 min with a chemical purity of >98%.

The effect of the FPI-rule on the suitability of the KBS-3H concept

International Nuclear Information System (INIS)

Pekkarinen, M.

2014-12-01

In the KBS-3H design the spent nuclear fuel canisters are placed in horizontal depositions drifts. In this work the degree of utilization, the number of placed canisters, the number of canisters in critical positions, and other related figures are estimated with Monte Carlo simulations. For comparison, the results are also computed for the KBS-3V design, where the canisters are placed in vertical deposition holes below tunnels. Different FPI criteria are evaluated for their performance in detecting the critical positions, i.e. positions where the super-container of the canister intersects a fracture with diameter over 150 m. Two different canister placement algorithms are considered, and the simulations are run with and without considering modeled site scale fault zones. The simple FPI criterion, where all full perimeter intersections are considered critical, was found overly conservative, leading to low degree of utilization (46-73 %). FPI criteria that require that a fracture is an FPI in multiple drifts are better, although the degree of utilization is still poor at the outskirts of the repository. The number of canisters in critical position is also 2-20 times as large as with the simple criterion. In KBS-3V design the criteria reject a much smaller number of canister positions, because the number of FPIs is smaller. A larger number of canisters are placed in critical position in KBS-3V design, because the FPC cannot detect the large fractures below the tunnels. (orig.)

Two cases of colorectal cancer complicating radiation enterocolitis

International Nuclear Information System (INIS)

Honma, Kaneatsu; Muto, Yoshihiro; Kusano, Toshiomi; Tokumine, Akio; Okushima, Norihiko; Tamashiro, Tetsuo

1993-01-01

A 74-year-old woman presented with bowel movement disorder. She had received radiation therapy with 60 Gy for uterine cervical cancer approximately 20 years before. Barium enema and colonofiberscopy revealed radiation enterocolitis. Thereafter, the patient was admitted to the hospital due to stricture of the sigmoid colon and an increased CEA and was diagnosed as having Borrmann II type colorectal well differentiated adenocarcinoma. Histological examination revealed stage I with no associated lymph node metastases. She is alive 3 years and 10 month after surgery. The other patient was a 65 year-old woman with a history of cervical cancer. Twenty-one years after combined hysterectomy and postoperative external irradiation of 45 Gy, the patient presented with melena. Detailed examination revealed colorectal adenocarcinoma. Simultaneously, barium enema revealed radiation enterocolitis. At surgery, intrapelvic area was found to be frozen due to irradiation. She has no evidence of metastasis 2 years after surgery. As can be shown in the two patients, patients developing radiation enterocolitis should be followed up periodically for the early detection of coexistent colorectal cancer. (N.K.)

Necrotizing enterocolitis - review of 34 cases

International Nuclear Information System (INIS)

Goncalves, E.G.; Abbud, E.A.; Duarte, F.B.

1990-01-01

Thirty-four cases of neonatal necrotizing enterocolitis are reviewed. The authors took into consideration the actual criteria of classification, and demonstrated the pre-disposal factors and the correlation between clinical and roentgenographics findings in this condition. (author)

Fluorine-18 labelling using [18F]FPyME of a small-glyco drug for potential applications in oncology

International Nuclear Information System (INIS)

Kuhnast, B.; Boisgard, R.; Hinnen, F.; Tavitian, B.; Dolle, F.; El Hadri, A.; Richard, S.; Caravano, A.; Petitou, M.

2011-01-01

Complete text of publication follows: Objectives: Proteoglycans, among which heparan sulfates (HS), are involved in many of the physiopathological steps of tumour development. Through their interaction with target proteins which regulate cell proliferation, migration, adhesion and invasion, HS play a crucial role in tumour angiogenesis and metastasis. Fully synthetic HS-mimetic oligosaccharides, also called small-glyco drugs, can be prepared and their affinity and inhibition profiles can be finely tuned according to the chemical substitutions. Access to these small-glyco drugs labeled with a positron emitter would be highly valuable in PET imaging not only for their pharmacological evaluation in vivo but also for a better understanding of tumour development. Prosthetic labeling is an efficient and reliable methodology that gives access to radiolabeled biological macromolecules. It consists in the preparation of a low molecular weight reagent bearing the radioactive isotope followed by its conjugation with the desired macromolecule. This strategy is particularly convenient when fluorine-18 is considered. Numerous prosthetic reagents have been designed among which [ 18 F]FPyME (a fluoro-pyridine-based maleimide reagent) for a selective conjugation with sulfhydryl functions borne by the macromolecules. In the present contribution, fluorine-18 labeling of the small-glyco drug EP80043 (c-2) via prosthetic labeling with [ 18 F]FPyME of the corresponding sulphated octa-saccharide, functionalized with a sulfhydryl function (2), is reported. Methods: [ 18 F]FPyME was prepared using a three-step radiochemical pathway, HPLC-purified and freed from HPLC solvents as already reported. The target octa-saccharide 2 was first synthesized as its acetylated derivative 1 to avoid intermolecular disulfide bridge formation. Prior to conjugation with [ 18 F]FPyME, 1 mg of 1 dissolved in PBS (0.1 M, pH 7.5, 100 μL) was treated with a 50 mM solution of hydroxylamine in PBS (100 μL) for

Radiological Findings in Two Patients with Cow's Milk Allergic Enterocolitis

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Kouji Masumoto

2004-07-01

Full Text Available Cow's milk allergic enterocolitis is a common paediatric gastrointestinal disease. However, radiological findings from contrast studies have rarely been reported. We report two Japanese neonate patients with cow's milk allergic enterocolitis with vomiting, bloody stool and unique findings on upper gastrointestinal contrast study. Upper gastrointestinal contrast study showed folds of the small intestine to be thickened, such as in spasm, or ribbonlike cords in both cases. There was also poor passage from the duodenum to the jejunum in one case. In addition, there were spastic findings on lower gastrointestinal contrast study in part of the large colon in one case. Clinicians, including paediatric surgeons, treating neonates with bloody stool and/or vomiting should thus be aware that unique findings on upper and lower gastrointestinal contrast studies may be useful in suspecting cases with cow's milk allergic enterocolitis.

Enterocolic lymphocytic phlebitis: statistical analysis of histology features in viable and ischemic bowel.

Science.gov (United States)

Medlicott, Shaun A C; Guggisberg, Kelly A; DesCôteaux, Jean-Gaston; Beck, Paul

2006-07-01

Enterocolic lymphocytic phlebitis is a rare cause of segmental ischemic enterocolitis. This artery-sparing transmural vasculitis is classically a circumferential phlebitis with perivenular lymphocyte cuffing and thrombi in the absence of systemic manifestations. Myointimal hyperplasia may represent a chronic phase of enterocolic lymphocytic phlebitis. Subclinical or early stage enterocolic lymphocytic phlebitis is not well delineated. We analyzed 600 submucosal and subserosal veins from both ischemic and intact bowel segments to discern if vascular morphology varied between sites. Crescentic and circumferential lymphocytic phlebitis is more common in viable bowel than in the ischemic segment. A nonsignificant trend was found for increased crescentic morphology between intact bowel remote from the ischemic focus compared with that adjacent to the ischemic focus. Hallmarks of ischemic bowel are necrotizing phlebitis and thrombi formation. Thrombophlebitis morphology is distinctly different in viable and ischemic bowel, changing from the classic lymphocytic to necrotizing lesions respectively.

Fatores associados à enterocolite necrosante Factors associated with necrotizing enterocolitis

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Maria Teresa Campos Vieira

2003-04-01

Full Text Available OBJETIVO: avaliar as formas de apresentação clínica da enterocolite necrosante neonatal e os fatores associados à doença. MÉTODOS: estudo retrospectivo dos casos de enterocolite necrosante neonatal (ECN (n = 56 ocorridos na UTI Neonatal Lagoa, entre dezembro de 1986 e julho de 1992. O diagnóstico e estadiamento da doença seguiram o critério de Bell modificado. Foram avaliados o diagnóstico e a evolução de todos os casos. Posteriormente, foram selecionados os casos de enterocolite grau II e III (n =44 e comparados com um grupo controle (n = 44, selecionado pelo peso de nascimento (± 250g e época de internação (± 2 semanas. Para a análise estatística, foi considerado significante p OBJECTIVE: to evaluate the features of clinical presentation of neonatal necrotizing enterocolitis and its associated factors. METHODS: retrospective study of the cases of neonatal necrotizing enterocolitis (n = 56 diagnosed at Neonatal Intensive Care Unit Lagoa (NICU, between December 1986 and July 1992. Diagnosis and stages of the disease followed the modified Bell's criteria. Diagnosis and follow-up of all cases were evaluated. The cases of enterocolitis - degrees II and III (n = 44 - were subsequently selected and compared to a case-control group (n = 44, selected according to birthweight ( 250 g and hospitalization period ( 2 weeks. The statistically significant analysis was considered as p < 0.05. RESULTS: out of 2,447 newborns admitted to the NICU, 56 (2.3% presented enterocolitis. Mean weight was 1908.5 g; mean gestational age was 35 weeks and 1 day; mean period for diagnosis was 10.7 days; 51 (91.1% patients were fed before diagnosis; 18 (32.1% needed urgent surgery; nine (16.9% hemocultures were positive; 10 (17.8% patients died. Four clinical standards were observed: fulminant, acute with pneumatosis, insidious and suspect. Comparatively to the case-control group, three factors were significantly associated with enterocolitis: apnea (p = 0

Management of necrotizing enterocolitis: experience at a tertiary ...

African Journals Online (AJOL)

that can predict the need for surgical management. Ann. Pediatr Surg ... Keywords: C-reactive protein, necrotizing enterocolitis, thrombocytopenia. aPediatric Surgery Unit .... between the use of eye drops and the development of. NEC.

[Risk factors of necrotizing enterocolitis].

Science.gov (United States)

Tapia-Rombo, C A; Velasco-Lavín, M R; Nieto-Caldelas, A

1993-09-01

The purpose of the present study is to compare risk factors of necrotizing enterocolitis (NEC) between two group: group A, newborns with the disease and group B, newborns with other diseases different from NEC, in order to know if these risk factors are more frequent or not in the first group. We assessed the clinical records of all the patients hospitalized in the Neonatal Intensive Care Unit and Neonatology Service of the La Raza General Hospital between 1987 and 1991 with the diagnosis of NEC. They were compared with 65 clinical records chosen at random of patients hospitalized in the same Unit with other diagnosis at the same time, and who were discharged by improvement or deceased. In all of them were look for known risk factors for NEC generally accepted such as: prematurity, neonatal asphyxia, poliglobulia, cyanotic congenital heart disease, patent ductus arteriosus, respiratory distress syndrome, catheterization of umbilical vessels, early feeding of elevated formula increases, exchange exchange transfusion, hypoxic ischemic encephalopathy, infection, etc. Just 25 records of the possible 50 with the diagnosis of NEC full filled inclusion criteria. There were no statistically significant difference in weight, sex, mortality and known risk factors of NEC between both groups. Were concluded that NEC is a disease of unknown etiology that should be studied more thoroughly. The known risk factors must be avoided because the patient susceptibility probably play an important role.

CLMP is required for intestinal development, and loss-of-function mutations cause congenital short-bowel syndrome

NARCIS (Netherlands)

Van Der Werf, Christine S.; Wabbersen, Tara D.; Hsiao, Nai-Hua; Paredes, Joana; Etchevers, Heather C.; Kroisel, Peter M.; Tibboel, Dick; Babarit, Candice; Schreiber, Richard A.; Hoffenberg, Edward J.; Vekemans, Michel; Zeder, Sirkka L.; Ceccherini, Isabella; Lyonnet, Stanislas; Ribeiro, Ana S.; Seruca, Raquel; Meerman, Gerard J. Te; van Ijzendoorn, Sven C. D.; Shepherd, Iain T.; Verheij, Joke B. G. M.; Hofstra, Robert M. W.

BACKGROUND & AIMS: Short-bowel syndrome usually results from surgical resection of the small intestine for diseases such as intestinal atresias, volvulus, and necrotizing enterocolitis. Patients with congenital short-bowel syndrome (CSBS) are born with a substantial shortening of the small

Microvillus Inclusion Disease Associated with Necrotizing Enterocolitis in a Premature Infant

Directory of Open Access Journals (Sweden)

Ersin Sayar

2014-11-01

Full Text Available Microvillus inclusion disease is one of the congenital diarrheal disorders characterized by the appearance of inclusion bodies on the intestinal epithelium. To date there are a few cases and also a few other associated finding reports related to this life-threatening disease in literature. In this report, we present a premature infant with microvillus inclusion disease that was associated with necrotizing enterocolitis. Thus, we should be aware of the appearance of necrotizing enterocolitis in patients with microvillus inclusion disease, especially when contributing factors are present.

Necrotizing enterocolitis is associated with earlier achievement of enteral autonomy in children with short bowel syndrome.

Science.gov (United States)

Sparks, Eric A; Khan, Faraz A; Fisher, Jeremy G; Fullerton, Brenna S; Hall, Amber; Raphael, Bram P; Duggan, Christopher; Modi, Biren P; Jaksic, Tom

2016-01-01

Necrotizing enterocolitis (NEC) remains one of the most common underlying diagnoses of short bowel syndrome (SBS) in children. The relationship between the etiology of SBS and ultimate enteral autonomy has not been well studied. This investigation sought to evaluate the rate of achievement of enteral autonomy in SBS patients with and without NEC. Following IRB approval, 109 patients (2002-2014) at a multidisciplinary intestinal rehabilitation program were reviewed. The primary outcome evaluated was achievement of enteral autonomy (i.e. fully weaning from parenteral nutrition). Patient demographics, primary diagnosis, residual small bowel length, percent expected small bowel length, median serum citrulline level, number of abdominal operations, status of the ileocecal valve (ICV), presence of ileostomy, liver function tests, and treatment for bacterial overgrowth were recorded for each patient. Median age at PN onset was 0 weeks [IQR 0-0]. Median residual small bowel length was 33.5 cm [IQR 20-70]. NEC was present in 37 of 109 (33.9%) of patients. 45 patients (41%) achieved enteral autonomy after a median PN duration of 15.3 [IQR 7.2-38.4]months. Overall, 64.9% of patients with NEC achieved enteral autonomy compared to 29.2% of patients with a different primary diagnosis (p=0.001, Fig. 1). Patients with NEC remained more likely than those without NEC to achieve enteral autonomy after two (45.5% vs. 12.0%) and four (35.7% vs. 6.3%) years on PN (Fig. 1). Logistic regression analysis demonstrated the following parameters as independent predictors of enteral autonomy: diagnosis of NEC (pChildren with SBS because of NEC have a significantly higher likelihood of fully weaning from parenteral nutrition compared to children with other causes of SBS. Additionally, patients with NEC may attain enteral autonomy even after long durations of parenteral support. Copyright © 2016 Elsevier Inc. All rights reserved.

Neutropenic enterocolitis (typhlitis) associated with infectious mononucleosis

International Nuclear Information System (INIS)

Sigirci, Ahmet; Akinci, Aysehan; Oezgen, Uensal; Oezen, Metehan

2006-01-01

Neutropenic enterocolitis (typhlitis) is an unusual acute complication of neutropenia, most often associated with leukaemia and lymphoma and characterized by segmental caecal and ascending colonic ulceration that may progress to necrosis, perforation, and septicaemia. We present a unique case of an 8-year-old girl with recently diagnosed infectious mononucleosis having findings consistent with typhlitis on abdominal CT. (orig.)

Neutropenic enterocolitis (typhlitis) associated with infectious mononucleosis

Energy Technology Data Exchange (ETDEWEB)

Sigirci, Ahmet [Inonu University School of Medicine, Department of Radiology, Turgut Ozal Medical Centre, Malatya (Turkey); Akinci, Aysehan; Oezgen, Uensal; Oezen, Metehan [Inonu University School of Medicine, Department of Paediatrics, Turgut Ozal Medical Centre, Malatya (Turkey)

2006-02-01

Neutropenic enterocolitis (typhlitis) is an unusual acute complication of neutropenia, most often associated with leukaemia and lymphoma and characterized by segmental caecal and ascending colonic ulceration that may progress to necrosis, perforation, and septicaemia. We present a unique case of an 8-year-old girl with recently diagnosed infectious mononucleosis having findings consistent with typhlitis on abdominal CT. (orig.)

Association of Shah-Waardenburgh syndrome: a review of 6 cases.

Science.gov (United States)

Jan, Iftikhar A; Stroedter, Lutz; Haq, Anwaar-ul; Din, Zaheer-ud

2008-04-01

Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. The aim of the article is to study the relative frequency of associations in 6 consecutive cases of SWS. A review of 6 consecutive patients with SWS was performed to study the frequency of various components of the syndrome. Six patients had features of SWS. All patients had HD; of these, 3 had rectosigmoid HD, whereas 3 had extended HD. All patients had white forelock of hairs with skin depigmentation. One patient had sensorineural deafness, whereas other babies were less than 1 year, and thus, full evaluation of hearing deficiency was not assessed. Three patients had blue eyes, whereas other babies had normal iris pigmentation. Skin depigmentation was noted in 5 of the 6 patients. Three babies were seriously malnourished and showed higher association of enterocolitis. Shah-Waardenburg syndrome is an uncommon association of HD. Depigmentation with a white forelock and skin manifestations are common, whereas blue iris, long segment disease, and enterocolitis are present in nearly half of the patients.

Surgery for necrotising enterocolitis : primary anastomosis or enterostomy?

NARCIS (Netherlands)

Hofman, FN; Bax, NMA; van der Zee, DC

The ideal surgical management of neonates with necrotising enterocolitis (NEC) is still a matter of debate. The purpose of this study was to compare the results of bowel resection with primary anastomosis with the results of bowel resection with enterostomy. Sixty-three neonates with NEC had a bowel

Factores de riesgo asociados a la mortalidad por enterocolitis necrotizante

Directory of Open Access Journals (Sweden)

Vivian R. Mena Miranda

1998-06-01

Full Text Available La enterocolitis necrotizante es una urgencia gastrointestinal de causa multifactorial muy relacionada con el neonato pretérmino. Su elevada mortalidad radica en la falta de prevención por el médico a cualquier nivel de atención y a su diagnóstico tardío en los grupos de riesgo. Se realizó un estudio retrospectivo de los 63 pacientes fallecidos por enterocolitis necrotizante durante un período de 25 años en el Hospital Pediátrico Docente de Centro Habana, donde se encontró que el 71,4 % de los afectados era de la raza blanca y el 68,2 % del sexo masculino. La edad más frecuente se encontró en los menores de 3 meses de edad (36,5 % y el 46 % del total de la muestra estudiada tuvo un peso al nacer inferior a los 1 500 g. La prematuridad apareció asociada en el 55,5 % de los fallecidos y el 65 % tuvo lactancia mixta desde el momento de nacimiento.Necrotizing enterocolitis is a gastrointestinal urgency of multifactorial cause taht is closely connected with the preterm neonatus. Its high mortality results from the lack of prevention on the part of the physician at any level of attention and from its late diagnosis in the risk groups. A retrospective study of 63 patients who died of necrotizing enterocolitis during a period of 25 years at the Pediatric Teaching Hospital of Central Havana was conducted. It was found that 71.4 % of the affected were white and 68.2 % were males. It was more frequent among those under 3 months (36.5 %. 46 % of the total of the sample studied had a birth weight of less than 1 500 g. Prematurity appeared associated in 55 % of the dead, whereas 65 % had a mixed lactation since their birth.

Enterocolitis necrozante

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Sandra Janeth Prada Buitrago

2008-04-01

Full Text Available La enterocolitis necrozante (ECN es un síndrome deetiología multifactorial caracterizado por necrosiscoagulativa a nivel del tejido intestinal, que lleva a infecciónbacteriana de la pared ulcerada, comprometiendoprincipalmente el ileon terminal y el colon. Se asocia conalgunos factores de riesgo como: prematuridad, bajo pesoal nacer, hipoxia neonatal y alimentación enteral temprana.Los primeros signos y síntomas de ECN suelen serinespecíficos y comprenden hallazgos clínicos deenfermedad séptica y enfermedad gastrointestinal. Seclasifica en estadios según los criterios de Bell y cols, queabarcan desde la enfermedad subclínica (IA hasta la ECNavanzada (IIIB; esta clasificación permite evaluar criteriosradiológicos, clínicos y de laboratorio. El tratamientodepende del estadio clínico en el cual se encuentre elpaciente. El abordaje terapéutico incluye soporte conlíquidos endovenosos, oxigeno, descompresión gástrica,correcto uso de antibióticos y en ciertos casos intervenciónquirúrgica.

Extensively Hydrolyzed Formula (MA-mi Induced Exacerbation of Food Protein-Induced Enterocolitis Syndrome (FPIES in a Male Infant

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Tomoyuki Kabuki

2007-01-01

Discussion: MA-mi is likely to be used increasingly for allergic infants, but it is not necessarily a substitute for other hydrolyzed milk formulae in all cases, and care should be taken regarding its use and possible misuse.

Association of cord blood digitalis-like factor and necrotizing enterocolitis.

LENUS (Irish Health Repository)

Graves, Steven W

2014-04-01

Endogenous digoxin-like factor (EDLF) has been linked to vasoconstriction, altered membrane transport, and apoptosis. Our objective was to determine whether increased EDLF in the cord sera of preterm infants was associated with an increased incidence of necrotizing enterocolitis (NEC).

FPI observations of nighttime mesospheric and thermospheric winds in China and their comparisons with HWM07

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W. Yuan

2013-08-01

Full Text Available We analyzed the nighttime horizontal neutral winds in the middle atmosphere (~ 87 and ~ 98 km and thermosphere (~ 250 km derived from a Fabry–Perot interferometer (FPI, which was installed at Xinglong station (40.2° N, 117.4° E in central China. The wind data covered the period from April 2010 to July 2012. We studied the annual, semiannual and terannual variations of the midnight winds at ~ 87 km, ~ 98 km and ~ 250 km for the first time and compared them with Horizontal Wind Model 2007 (HWM07. Our results show the following: (1 at ~ 87 km, both the observed and model zonal winds have similar phases in the annual and semiannual variations. However, the HWM07 amplitudes are much larger. (2 At ~ 98 km, the model shows strong eastward wind in the summer solstice, resulting in a large annual variation, while the observed strongest component is semiannual. The observation and model midnight meridional winds agree well. Both are equatorward throughout the year and have small amplitudes in the annual and semiannual variations. (3 There are large discrepancies between the observed and HWM07 winds at ~ 250 km. This discrepancy is largely due to the strong semiannual zonal wind in the model and the phase difference in the annual variation of the meridional wind. The FPI annual variation coincides with the results from Arecibo, which has similar geomagnetic latitude as Xinglong station. In General, the consistency of FPI winds with model winds is better at ~ 87 and ~ 98 km than that at ~ 250 km. We also studied the seasonally and monthly averaged nighttime winds. The most salient features include the following: (1 the seasonally averaged zonal winds at ~ 87 and ~ 98 km typically have small variations throughout the night. (2 The model zonal and meridional nighttime wind variations are typically much larger than those of observations at ~ 87 km and ~ 98 km. (3 At ~ 250 km, model zonal wind compares well with the observation in the winter. For spring and

Assessing patient report of function: content validity of the Functional Performance Inventory-Short Form (FPI-SF in patients with chronic obstructive pulmonary disease (COPD

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Leidy NK

2012-08-01

Full Text Available Nancy Kline Leidy,1 Alan Hamilton,2 Karin Becker31Outcomes Research, United BioSource Corporation, Bethesda, MD, USA; 2Medical Department, Boehringer Ingelheim (Canada Ltd, Burlington, ON, Canada; 3Global Health Economics and Outcomes Research, Boehringer Ingelheim GmbH, Ingelheim am Rhein, GermanyPurpose: The performance of daily activities is a major challenge for people with chronic obstructive pulmonary disease (COPD. The Functional Performance Inventory (FPI was developed based on an analytical framework of functional status and qualitative interviews with COPD patients describing these difficulties. The 65-item FPI was reduced to a 32-item short form (SF through a systematic process of qualitative and quantitative item reduction and formatted for greater clarity and ease of use. This study examined the content validity of the reduced, reformatted form of the instrument, the FPI-SF.Patients and methods: Qualitative cognitive interviews were conducted with COPD patients recruited from three geographically diverse pulmonary clinics in the United States. Interviews were designed to assess respondent interpretation of the instrument, evaluate clarity and ease of completion, and identify any new activities participants found important and difficult to perform that were not represented by the existing items.Results: Twenty subjects comprised the sample; 12 (60% were male, 14 (70% were Caucasian, the mean age was 63.0 ± 11.3 years, 12 (60% were retired, the mean forced expiratory volume in 1 second (FEV1 was 1.5 ± 0.5 L, and the mean percent predicted FEV1 was 48.4% ± 13.1%. Participants understood the FPI-SF as intended, including instructions, items, and response options. Two minor formatting changes were suggested to improve clarity of presentation. Participants found the content of the FPI-SF to be comprehensive, with items covering activities they felt were important and often difficult to perform.Conclusion: These results, together with

Participation of some campylobacter species in the etiology of enterocolitis

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Otašević Marica M.

2004-01-01

Full Text Available Background. In recent decades, medical community has increasingly been calling attention to the importance of Campylobacter as an disease-causing agent in humans. Nowdays, Campylobacter jejuni (C. jejuni is known as the most frequent bacterial cause of diarrhea worldwide. Epidemiological differences of the infections caused by Campylobacter, present in the developed and the developing countries, are attributed to the differences of the types of virulence. Due to the specificity, and the demanding features of Campylobacter, as well as poorly equipped microbiological laboratories, campylobacteriosis is insufficiently studied in our country. This investigation aimed to determine the participation of some Campylobacter species in the etiology of diarrheal diseases in our population. Methods. The four-years continuous monitoring of Campylobacter presence was performed in the faeces of 12 605 patients with enterocolitis. The control group included 5 774 examinees of healthy children and youth. Faeces samples were cultivated on Skirrow's selective medium, and further incubated according to effective methodology for Campylobacter. Identification of strains was based on morphological, cultural and physiologic features of strains (oxidase test, catalase test, susceptibility to nalidixic acid, and hypurate hydrolysis. As a statistical method, for data processing, c2 test and Fisher’s exact test were used. Results. Campylobacter was proven in 3.86% of enterocolitis patients, and in 0.71% of healthy population. Out of 518 Campylobacter isolates, 86.48% belonged to enterocolitis outpatients, and 13,51% to inpatients. Predominant symptoms of the disease were diarrhea (81.83%, increased temperature (34.71%, vomiting (19.77%, and stomach pain (15.17%. The diseased were predominantly infants in the first year of life. Out of 300 Campylobacter isolates, 75% were identified as Campylobacer jejuni, 23% as Campylobacter coli (C. coli, and 2% as Campylobacter lari

Características de la enterocolitis necrotizante en neonatos Instituto Nacional Materno Perinatal 2010 - 2014

OpenAIRE

Anicama Elias, Erika Lucila

2017-01-01

Objetivo: Conocer las características de la enterocolitis necrotizante en neonatos del Instituto Nacional Materno Perinatal de Lima, 2010 – 2014 Método: Investigación descriptiva, observacional, transversal, retrospectiva, diseño no experimental. La población constituida por los 31 neonatos con diagnóstico de enterocolitis necrotizante hospitalizada en la Unidad de Cuidados Intensivos del Instituto Nacional Materno Perinatal durante los años 2010-2014. Resultados: La variable prematuridad est...

Gut bacteria are rarely shared by co-hospitalized premature infants, regardless of necrotizing enterocolitis development

Science.gov (United States)

Raveh-Sadka, Tali; Thomas, Brian C; Singh, Andrea; Firek, Brian; Brooks, Brandon; Castelle, Cindy J; Sharon, Itai; Baker, Robyn; Good, Misty; Morowitz, Michael J; Banfield, Jillian F

2015-01-01

Premature infants are highly vulnerable to aberrant gastrointestinal tract colonization, a process that may lead to diseases like necrotizing enterocolitis. Thus, spread of potential pathogens among hospitalized infants is of great concern. Here, we reconstructed hundreds of high-quality genomes of microorganisms that colonized co-hospitalized premature infants, assessed their metabolic potential, and tracked them over time to evaluate bacterial strain dispersal among infants. We compared microbial communities in infants who did and did not develop necrotizing enterocolitis. Surprisingly, while potentially pathogenic bacteria of the same species colonized many infants, our genome-resolved analysis revealed that strains colonizing each baby were typically distinct. In particular, no strain was common to all infants who developed necrotizing enterocolitis. The paucity of shared gut colonizers suggests the existence of significant barriers to the spread of bacteria among infants. Importantly, we demonstrate that strain-resolved comprehensive community analysis can be accomplished on potentially medically relevant time scales. DOI: http://dx.doi.org/10.7554/eLife.05477.001 PMID:25735037

Surgical management of radiation enterocolitis

International Nuclear Information System (INIS)

Ieda, Katsuyuki; Katsumi, Masaharu; Ura, Shinzoh

1980-01-01

We reviewed 17 cases of severe radiation enterocolitis caused by tele-cobalt treatment for pelvic malignancies. They consisted of six males and nine females, ranging from 32 to 77 years old. The duration between the completion of radiation and the onset of symptoms varied from two months to ten years. Only two cases of them were treated conservatively and the other 15 cases were managed surgically. Six cases of the latter underwent an urgent surgery because of severe obstructive symptoms. Totally, 19 injuries to the intestine were revealed. The ileum was involved in eight patients, the rectum in seven and the sigmoid colon in four. Operative procedures carried out were intestinal resection with primary anastomosis in seven, colostomy alone in six and bypass operation in three. The resected segments of the ileum measured 40 cm long in two and 70 cm, 90 cm and 100 cm long in three respectively and the resected segments of the colon measured 15 cm and 45 cm long respectively. Three out of the seven cases with bowel resection were reconstructed with Gambee's single layer anastomosis and four with Albert-Lembert's two layer anastomosis. Type of anastomosis was end-to-end in six and end-to-side in one. Three cases underwent bypass operations because the injured intestines were densely adhered to the surroundings. Only one minor leakage occurred in seven primary anastomosis. Radiation doses ranged from 3000 to 9300 R. There was no relation between doses and severity of damage, clinical symptoms and site of injuries. There was no malignant findings around the damaged intestine. Many of the literatures report a high anastomotic leak in radiation enterocolitis, primary anastomosis can be carried out more safely if wide resection and reasonable anastomosis are performed. (author)

Streptococcus bovis septicemia and meningitis associated with chronic radiation enterocolitis

International Nuclear Information System (INIS)

Jadeja, L.; Kantarjian, H.; Bolivar, R.

1983-01-01

We describe the first patient with simultaneous S bovis septicemia and meningitis associated with chronic radiation enterocolitis. This case underlines the value of a thorough gastrointestinal evaluation of all patients with S bovis infection, and the need for a neurologic investigation even with minor neurologic manifestations

Enterocolic lymphocytic phlebitis as a newly recognized manifestation of IgG4-related disease.

Science.gov (United States)

Laco, Jan; Örhalmi, Július; Bártová, Jolana; Zimandlová, Dana

2015-04-01

Herein we present a case of a 65-year-old woman with enterocolic lymphocytic phlebitis (ELP) who presented with anemic syndrome and in whom severe stenosis of the right flexure of large bowel was detected. The microscopic examination revealed fibrosis of the submucosa and lymphoplasmacytic phlebitis of small veins and venules, whereas arteries were spared. There were 110 IgG4-positive and 160 IgG-positive plasma cells in 1 high-power field, respectively, with corresponding IgG4/IgG ratio of 0.69. The IgG4 serum level was 2.42 g/L. According to the currently proposed criteria, this ELP case is the first that may be diagnosed as definite IgG4-related disease (IgG4-RD). Although based on the sole case description, taken together with a recent review and a case report, we presume that a subset of ELPs is a manifestation of IgG4-RD. © The Author(s) 2014.

Fluorine-18 labelling of a novel series of chimeric, mdm2 oncogene targeting, peptide-pna oligomers using [18F]FPyME

International Nuclear Information System (INIS)

Kuhnast, B.; Hinnen, F.; Boisgard, R.; Tavitian, B.; Dolle, F.; Nielsen, P.

2011-01-01

Complete text of publication follows: Peptide nucleic acids (PNAs) form a unique class of synthetic macromolecules, originally designed as ligands for the recognition of double stranded DNA, where the deoxyribose phosphate backbone of original DNA is replaced by a pseudo-peptide N-(2-aminoethyl)glycyl backbone, while retaining the nucleobases of DNA. PNAs have already showed promising therapeutic potential as antisense and anti-gene agents and are inspiring the development of a variety of research and diagnostic assays, including their use as imaging tools. Within our intensive programs of development of oligonucleotide-based probes for PET-imaging, a novel series of chimeric peptide-PNA oligomers has been designed as complementary antisense probes targeting a specific 15-base sequence located at the intron-exon junction of the pre-mRNA of the murine double minute (mdm2) oncogene. This gene codes for a p53 interacting protein that represses p53 transcriptional activity, and appears to be over expressed in several tumor types including soft tissue sarcomas and osteosarcomas as well as breast tumors. For in vivo 3D-imaging purposes, all oligomers include a cysteine thus providing a sulfhydryl function permitting prosthetic conjugation with maleimide-based reagents such as AlexaFluor680 R (AF680) for optical fluorescence imaging and [ 18 F]FPyME (1-[3-(2-[ 18 F]fluoropyridin-3-yloxy)propyl]pyrrole-2, 5-dione), a prosthetic reagent labeled with the positron-emitter fluorine-18 for PET imaging, which latter work is presented herein. Methods: [ 18 F]FPyME was prepared using a three-step radiochemical pathway already reported and includes an HPLC-purification (semi-preparative SiO 2 Zorbax R Rx-SIL, Hewlett Packard). [ 18 F]FPyME was conjugated with the peptide-PNA oligomers (PNA3132, PNA3133, and PNA3135, 0.25-0.30 micro-moles) in 1/9 (v:v) mixture (1 mL) of DMSO and 0.1 M aq. PBS (pH 8) at room temperature for 15 min. The [ 18 F]FPyME-conjugated products (c-[ 18 F

Infants Operated on for Necrotizing Enterocolitis: Towards Evidence-Based Pain Guidelines

NARCIS (Netherlands)

N.J. Meesters (Naomi J.); M. van Dijk (Monique); C.A.J. Knibbe (Catherijne); C.M.G. Keyzer-Dekker (Claudia M.G.); D. Tibboel (Dick); S.H. Simons (Sinno)

2016-01-01

textabstractBackground: Necrotizing enterocolitis (NEC) is known as an extremely painful childhood condition. Objectives: The objective of this study was to explore pain management around NEC-related surgery in our neonatal intensive care unit (NICU) from a chart review of prospectively collected

Factores de riesgo asociados a la mortalidad por enterocolitis necrotizante

Directory of Open Access Journals (Sweden)

Vivian R. Mena Miranda

Full Text Available La enterocolitis necrotizante es una urgencia gastrointestinal de causa multifactorial muy relacionada con el neonato pretérmino. Su elevada mortalidad radica en la falta de prevención por el médico a cualquier nivel de atención y a su diagnóstico tardío en los grupos de riesgo. Se realizó un estudio retrospectivo de los 63 pacientes fallecidos por enterocolitis necrotizante durante un período de 25 años en el Hospital Pediátrico Docente de Centro Habana, donde se encontró que el 71,4 % de los afectados era de la raza blanca y el 68,2 % del sexo masculino. La edad más frecuente se encontró en los menores de 3 meses de edad (36,5 % y el 46 % del total de la muestra estudiada tuvo un peso al nacer inferior a los 1 500 g. La prematuridad apareció asociada en el 55,5 % de los fallecidos y el 65 % tuvo lactancia mixta desde el momento de nacimiento.

Abdominal ultrasonographic findings in typhoid fever: a comparison between typhoid patients and those with non-typhoidal Salmonella and Campylobacter jejuni enterocolitis.

Science.gov (United States)

Kobayashi, Akira; Adachi, Yasuo; Iwata, Yoshinori; Sakai, Yoshiyuki; Shigemitu, Kazuaki; Todoroki, Miwako; Ide, Mituru

2012-03-01

Typhoid fever is a major health problem in many developing countries and its clinical features are similar to other types of bacterial enterocolitis. Definitive diagnosis by blood culture requires several days and is often unfeasible to perform in developing countries. More efficient and rapid diagnostic methods for typhoid are needed. We compared the pathological changes in the bowel and adjacent tissues of patients having typhoid fever with those having bacterial enterocolitis using ultrasonography. A characteristic of patients with non-typhoidal Salmonella and Campylobacter jejuni enterocolitis was mural thickening of the terminal ileum; only mild mural swelling or no swelling was observed in patients with typhoid fever. Mesenteric lymph nodes in patients with typhoid fever were significantly more enlarged compared to patients with other types of bacterial enterocolitis. Our findings suggest typhoid fever is not fundamentally an enteric disease but rather resembles mesenteric lymphadenopathy and ultrasound is a promising modality for diagnosing typhoid fever in developing countries.

Intestinal upregulation of melanin-concentrating hormone in TNBS-induced enterocolitis in adult zebrafish.

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Brenda M Geiger

Full Text Available BACKGROUND: Melanin-concentrating hormone (MCH, an evolutionarily conserved appetite-regulating neuropeptide, has been recently implicated in the pathogenesis of inflammatory bowel disease (IBD. Expression of MCH is upregulated in inflamed intestinal mucosa in humans with colitis and MCH-deficient mice treated with trinitrobenzene-sulfonic acid (TNBS develop an attenuated form of colitis compared to wild type animals. Zebrafish have emerged as a new animal model of IBD, although the majority of the reported studies concern zebrafish larvae. Regulation MCH expression in the adult zebrafish intestine remains unknown. METHODS: In the present study we induced enterocolitis in adult zebrafish by intrarectal administration of TNBS. Follow-up included survival analysis, histological assessment of changes in intestinal architecture, and assessment of intestinal infiltration by myeloperoxidase positive cells and cytokine transcript levels. RESULTS: Treatment with TNBS dose-dependently reduced fish survival. This response required the presence of an intact microbiome, since fish pre-treated with vancomycin developed less severe enterocolitis. At 6 hours post-challenge, we detected a significant influx of myeloperoxidase positive cells in the intestine and upregulation of both proinflammatory and anti-inflammatory cytokines. Most importantly, and in analogy to human IBD and TNBS-induced mouse experimental colitis, we found increased intestinal expression of MCH and its receptor in TNBS-treated zebrafish. CONCLUSIONS: Taken together these findings not only establish a model of chemically-induced experimental enterocolitis in adult zebrafish, but point to effects of MCH in intestinal inflammation that are conserved across species.

Necrotising Enterocolitis and Systemic Inflammation: Placental Pathology and Haematological Markers of Mortality

LENUS (Irish Health Repository)

Baastad, S

2011-11-01

Necrotizing enterocolitis (NEC) is a devastating disease that affects premature neonates. Associated mortality has not changed appreciably over the past several decades. The underlying aetiology of NEC remains elusive, although bacterial colonization of the gut, formula feeding, and perinatal stress have been implicated as risk factors.\\r\

Diet- and colonization-dependent intestinal dysfunction predisposes to necrotizing enterocolitis in preterm pigs

DEFF Research Database (Denmark)

Sangild, Per T.; Siggers, Richard H.; Schmidt, Mette

2006-01-01

Background & Aims: Preterm birth and formula feeding are key risk factors associated with necrotizing enterocolitis (NEC) in infants, but little is known about intestinal conditions that predispose to disease. Thus, structural, functional, and microbiologic indices were used to investigate...

Sialylated galacto-oligosaccharides and 2'-fucosyllactose reduce necrotising enterocolitis in neonatal rats

NARCIS (Netherlands)

Autran, Chloe A.; Schoterman, Margriet H.C.; Jantscher-Krenn, Evelyn; Kamerling, Johannis P.; Bode, Lars

2016-01-01

Necrotising enterocolitis (NEC) is one of the most frequent and fatal intestinal disorders in preterm infants and has very limited treatment options. Breast-fed infants are at a 6-10-fold lower NEC risk than formula-fed infants, and we have previously shown that human milk oligosaccharides (HMO)

Characterization of Intestinal Microbiomes of Hirschsprung's Disease Patients with or without Enterocolitis Using Illumina-MiSeq High-Throughput Sequencing.

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Yuqing Li

Full Text Available Hirschsprung-associated enterocolitis (HAEC is a life-threatening complication of Hirschsprung's disease (HD. Although the pathological mechanisms are still unclear, studies have shown that HAEC has a close relationship with the disturbance of intestinal microbiota. This study aimed to investigate the characteristics of the intestinal microbiome of HD patients with or without enterocolitis. During routine or emergency surgery, we collected 35 intestinal content samples from five patients with HAEC and eight HD patients, including three HD patients with a history of enterocolitis who were in a HAEC remission (HAEC-R phase. Using Illumina-MiSeq high-throughput sequencing, we sequenced the V4 region of bacterial 16S rRNA, and operational taxonomic units (OTUs were defined by 97% sequence similarity. Principal coordinate analysis (PCoA of weighted UniFrac distances was performed to evaluate the diversity of each intestinal microbiome sample. The microbiota differed significantly between the HD patients (characterized by the prevalence of Bacteroidetes and HAEC patients (characterized by the prevalence of Proteobacteria, while the microbiota of the HAEC-R patients was more similar to that of the HAEC patients. We also observed that the specimens from different intestinal sites of each HD patient differed significantly, while the specimens from different intestinal sites of each HAEC and HAEC-R patient were more similar. In conclusion, the microbiome pattern of the HAEC-R patients was more similar to that of the HAEC patients than to that of the HD patients. The HD patients had a relatively distinct, more stable community than the HAEC and HAEC-R patients, suggesting that enterocolitis may either be caused by or result in a disruption of the patient's uniquely adapted intestinal flora. The intestinal microbiota associated with enterocolitis may persist following symptom resolution and can be implicated in the symptom recurrence.

Updating on gut microbiota and its relationship with the occurrence of necrotizing enterocolitis

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Michel Hosny

2017-06-01

Full Text Available Necrotizing enterocolitis (NEC remains a leading cause of morbidity and mortality, affecting primarily preterm neonates. The pathogenesis of this intestinal disease appears to be linked to the disruption or delay of bacterial colonization, termed gut dysbiosis. Intestinal immaturity, antibiotic use and hospital microbial environment are the main triggers of this pathological process. Conversely, gut symbiosis is made possible by the presence of beneficial and commensal bacterial species that protect the immature gut from opportunistic pathogens overgrowth and inflammation. Herein, we review the relationships between gut microbiota and NEC in preterm neonates. We also discuss the role of specific microorganisms belonging to the commensal microbiota, highlighting the possibility for a toxigenic mechanism involved in NEC pathogenesis. We conclude on the importance of interventions aimed at providing or restoring beneficial bacteria populations, in view to efficiently preventing or treating NEC. Keywords: Necrotizing enterocolitis, Gut microbiota, Dysbiosis, Toxins

Comparison of high-latitude thermospheric meridionalwinds II: combined FPI, radar and model Climatologies

Directory of Open Access Journals (Sweden)

E. M. Griffin

2004-03-01

Full Text Available The climatological behaviour of the thermospheric meridional wind above Kiruna, Sweden (67.4°N, 20.4°E has been investigated for seasonal and solar cycle dependence using six different techniques, comprising both model and experimental sources. Model output from both the empirical Horizontal Wind Model (HWM (Hedin et al., 1988 and the numerical Coupled Thermosphere and Ionosphere Model (CTIM are compared to the measured behaviour at Kiruna, as a single site example. The empirical International Reference Ionosphere (IRI model is used as input to an implementation of servo theory, to provide another climatology combining empirical input with a theoretical framework. The experimental techniques have been introduced in a companion paper in this issue and provide climatologies from direct measurements, using Fabry-Perot Interferometers (FPI, together with 2 separate techniques applied to the European Incoherent Scatter radar (EISCAT database to derive neutral winds. One of these techniques uses the same implementation of servo theory as has been used with the IRI model. Detailed comparisons for each season and solar activity category allow for conclusions to be drawn as to the major influences on the climatological behaviour of the wind at this latitude. Comparison of the incoherent scatter radar (ISR derived neutral winds with FPI, empirical model and numerical model winds is important to our understanding and judgement of the validity of the techniques used to derive thermospheric wind databases. The comparisons also test model performance and indicate possible reasons for differences found between the models. In turn, the conclusions point to possible improvements in their formulation. In particular it is found that the empirical models are over-reliant on mid-latitude data in their formulation, and fail to provide accurate estimates of the winds at high-latitudes.

Key words. Meteorology and atmospheric dynamics (thermospheric dynamics

Comparison of high-latitude thermospheric meridionalwinds II: combined FPI, radar and model Climatologies

Directory of Open Access Journals (Sweden)

E. M. Griffin

2004-03-01

Full Text Available The climatological behaviour of the thermospheric meridional wind above Kiruna, Sweden (67.4°N, 20.4°E has been investigated for seasonal and solar cycle dependence using six different techniques, comprising both model and experimental sources. Model output from both the empirical Horizontal Wind Model (HWM (Hedin et al., 1988 and the numerical Coupled Thermosphere and Ionosphere Model (CTIM are compared to the measured behaviour at Kiruna, as a single site example. The empirical International Reference Ionosphere (IRI model is used as input to an implementation of servo theory, to provide another climatology combining empirical input with a theoretical framework. The experimental techniques have been introduced in a companion paper in this issue and provide climatologies from direct measurements, using Fabry-Perot Interferometers (FPI, together with 2 separate techniques applied to the European Incoherent Scatter radar (EISCAT database to derive neutral winds. One of these techniques uses the same implementation of servo theory as has been used with the IRI model. Detailed comparisons for each season and solar activity category allow for conclusions to be drawn as to the major influences on the climatological behaviour of the wind at this latitude. Comparison of the incoherent scatter radar (ISR derived neutral winds with FPI, empirical model and numerical model winds is important to our understanding and judgement of the validity of the techniques used to derive thermospheric wind databases. The comparisons also test model performance and indicate possible reasons for differences found between the models. In turn, the conclusions point to possible improvements in their formulation. In particular it is found that the empirical models are over-reliant on mid-latitude data in their formulation, and fail to provide accurate estimates of the winds at high-latitudes. Key words. Meteorology and atmospheric dynamics (thermospheric dynamics

The timing of ostomy closure in infants with necrotizing enterocolitis: A systematic review

NARCIS (Netherlands)

A.E.C.J.M. Struijs (Marie-Chantal); C.E.J. Sloots (Pim); W.C.J. Hop (Wim); D. Tibboel (Dick); R.M.H. Wijnen (René)

2012-01-01

textabstractPurpose The optimal timing of ostomy closure is a matter of debate. We performed a systematic review of outcomes of early ostomy closure (EC, within 8 weeks) and late ostomy closure (LC, after 8 weeks) in infants with necro-tizing enterocolitis. Methods PubMed, EMbase, Web-of-Science,

The timing of ostomy closure in infants with necrotizing enterocolitis: a systematic review

NARCIS (Netherlands)

Struijs, M.C.; Sloots, C.E.J.; Hop, W.C.J.; Tibboel, D.; Wijnen, R.M.H.

2012-01-01

PURPOSE: The optimal timing of ostomy closure is a matter of debate. We performed a systematic review of outcomes of early ostomy closure (EC, within 8 weeks) and late ostomy closure (LC, after 8 weeks) in infants with necrotizing enterocolitis. METHODS: PubMed, EMbase, Web-of-Science, and Cinahl

First case report of myopericarditis linked to Campylobacter coli enterocolitis.

Science.gov (United States)

Moffatt, Cameron R M; Moloi, Soniah B; Kennedy, Karina J

2017-01-05

Campylobacter spp. are a common cause of mostly self-limiting enterocolitis. Although rare, pericarditis and myopericarditis have been increasingly documented as complications following campylobacteriosis. Such cases have occurred predominantly in younger males, and involved a single causative species, namely Campylobacter jejuni. We report the first case of myopericarditis following Campylobacter coli enterocolitis, with illness occurring in an immunocompetent middle-aged female. A 51-yo female was admitted to a cardiology unit with a 3-days history of chest pain. The woman had no significant medical history or risk factors for cardiac disease, nor did she report any recent overseas travel. Four days prior to the commencement of chest pain the woman had reported onset of an acute gastrointestinal illness, passing 3-4 loose stools daily, a situation that persisted at the time of presentation. Physical examination showed the woman's vital signs to be essentially stable, although she was noted to be mildly tachycardic. Laboratory testing showed mildly elevated C-reactive protein and a raised troponin I in the absence of elevation of the serum creatinine kinase. Electrocardiography (ECG) demonstrated concave ST segment elevations, and PR elevation in aVR and depression in lead II. Transthoracic echocardiogram (TTE) revealed normal biventricular size and function with no significant valvular abnormalities. There were no left ventricular regional wall motion abnormalities. No pericardial effusion was present but the pericardium appeared echodense. A diagnosis of myopericarditis was made on the basis of chest pain, typical ECG changes and troponin rise. The chest pain resolved and she was discharged from hospital after 2-days of observation, but with ongoing diarrhoea. Following discharge, a faecal sample taken during the admission, cultured Campylobacter spp. Matrix assisted laser desorption ionization time-of-flight (Bruker) confirmed the cultured isolate as C. coli

Functional Impairments at School Age of Children With Necrotizing Enterocolitis or Spontaneous Intestinal Perforation

NARCIS (Netherlands)

Roze, Elise; Ta, B.D.; van der Ree, Meike H.; Tanis, Jozien C.; van Braeckel, Koenraad N. J. A.; Hulscher, Jan B. F.; Bos, Arend F.

2011-01-01

We aimed to determine motor, cognitive, and behavioral outcome at school age of children who had either necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP). This case-control study included infants with NEC Bell's stage IIA onward, infants with SIP, and matched controls

[The correlation between personality characteristics and burnout syndrome in emergency ambulance workers].

Science.gov (United States)

Bergmueller, A; Zavgorodnii, I; Zavgorodnia, N; Kapustnik, W; Boeckelmann, I

The work in Emergency Medical Ambulance Service is one of the professions, which is accompanied by high psychological and emotional stress. The aim was to determine the risk of professional burnout syndrome of Emergency Medical Ambulance Service staff and the relationship between burnout syndrome and personality. Ninety-seven doctors (57 women and 40 men, aged 37.0±12.21) of Emergency Medical Ambulance Service were interviewed using the MBI-GS questionnaire and the Freiburg personality questionnaire (FPI). Correlation and regression analysis were used. Nine (11.5%) of respondents had a risk of burnout syndrome and 28 (35.9%) had some symptoms. In the group, aged 45 years and older, the risk of burnout syndrome was not identified. The staff of Emergency Medical Ambulance Service is characterized by stable personality features. It is necessary to identify the prevalence of burnout syndrome and also to identity the causes for its prevention and development of measures on the increase of personal stress resilience.

Non-Invasive Markers for Early Diagnosis and Determination of the Severity of Necrotizing Enterocolitis

NARCIS (Netherlands)

Thuijls, Geertje; Derikx, Joep P. M.; van Wijck, Kim; Zimmermann, Luc J. I.; Degraeuwe, Pieter L.; Mulder, Twan L.; Van der Zee, David C.; Brouwers, Hens A. A.; Verhoeven, Bas H.; van Heurn, L. W. Ernest; Kramer, Boris W.; Buurman, Wim A.; Heineman, Erik

Objectives: To improve diagnosis of necrotizing enterocolitis (NEC) by noninvasive markers representing gut wall integrity loss (I-FABP and claudin-3) and gut wall inflammation (calprotectin). Furthermore, the usefulness of I-FABP to predict NEC severity and to screen for NEC was evaluated. Methods:

Non-invasive markers for early diagnosis and determination of the severity of necrotizing enterocolitis.

NARCIS (Netherlands)

Thuijls, G.; Derikx, J.P.; Wijck, K. van; Zimmermann, L.J.; Degraeuwe, P.L.J.; Mulder, T.L.; Zee, D.C. van der; Brouwers, H.A.A.; Verhoeven, B.H.; Heurn, L.W.E. van; Kramer, B.W.; Buurman, W.A.; Heineman, E.

2010-01-01

OBJECTIVES: To improve diagnosis of necrotizing enterocolitis (NEC) by noninvasive markers representing gut wall integrity loss (I-FABP and claudin-3) and gut wall inflammation (calprotectin). Furthermore, the usefulness of I-FABP to predict NEC severity and to screen for NEC was evaluated. METHODS:

Characterization of Bacterial and Fungal Microbiome in Children with Hirschsprung Disease with and without a History of Enterocolitis: A Multicenter Study.

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Philip K Frykman

Full Text Available Development of potentially life-threatening enterocolitis is the most frequent complication in children with Hirschsprung disease (HSCR, even after definitive corrective surgery. Intestinal microbiota likely contribute to the etiology of enterocolitis, so the aim of this study was to compare the fecal bacterial and fungal communities of children who developed Hirschsprung-associated enterocolitis (HAEC with HSCR patients who had never had enterocolitis. Eighteen Hirschsprung patients who had completed definitive surgery were enrolled: 9 had a history of HAEC and 9 did not. Fecal DNA was isolated and 16S and ITS-1 regions sequenced using Next Generation Sequencing and data analysis for species identification. The HAEC group bacterial composition showed a modest reduction in Firmicutes and Verrucomicrobia with increased Bacteroidetes and Proteobacteria compared with the HSCR group. In contrast, the fecal fungi composition of the HAEC group showed marked reduction in diversity with increased Candida sp., and reduced Malassezia and Saccharomyces sp. compared with the HSCR group. The most striking finding within the HAEC group is that the Candida genus segregated into "high burden" patients with 97.8% C. albicans and 2.2% C. tropicalis compared with "low burden" patients 26.8% C. albicans and 73% C. tropicalis. Interestingly even the low burden HAEC group had altered Candida community structure with just two species compared to more diverse Candida populations in the HSCR patients. This is the first study to identify Candida sp. as potentially playing a role in HAEC either as expanded commensal species as a consequence of enterocolitis (or treatment, or possibly as pathobioants contributing to the pathogenesis of HAEC. These findings suggest a dysbiosis in the gut microbial ecosystem of HAEC patients, such that there may be dominance of fungi and bacteria predisposing patients to development of HAEC.

Comparison of high-latitude thermospheric meridional winds II: combined FPI, radar and model climatologies

Energy Technology Data Exchange (ETDEWEB)

Griffin, E.M.; Aruliah, A.; Mueller-Wodarg, I.C.F.; Aylward, A. [Atmospheric Physics Lab., Univ. Coll. London, London (United Kingdom)

2004-07-01

The climatological behaviour of the thermospheric meridional wind above Kiruna, Sweden (67.4 N, 20.4 E) has been investigated for seasonal and solar cycle dependence using six different techniques, comprising both model and experimental sources. Model output from both the empirical Horizontal Wind Model (HWM) (Hedin et al., 1988) and the numerical coupled thermosphere and ionosphere model (CTIM) are compared to the measured behaviour at kiruna, as a single site example. The empirical International Reference Ionosphere (IRI) model is used as input to an implementation of servo theory, to provide another climatology combining empirical input with a theoretical framework. The experimental techniques have been introduced in a companion paper in this issue and provide climatologies from direct measurements, using fabry-perot interferometers (FPI), together with 2 separate techniques applied to the European incoherent scatter radar (EISCAT) database to derive neutral winds. One of these techniques uses the same implementation of servo theory as has been used with the IRI model. Detailed comparisons for each season and solar activity category allow for conclusions to be drawn as to the major influences on the climatological behaviour of the wind at this latitude. Comparison of the incoherent scatter radar (ISR) derived neutral winds with FPI, empirical model and numerical model winds is important to our understanding and judgement of the validity of the techniques used to derive thermospheric wind databases. The comparisons also test model performance and indicate possible reasons for differences found between the models. In turn, the conclusions point to possible improvements in their formulation. In particular it is found that the empirical models are over-reliant on mid-latitude data in their formulation, and fail to provide accurate estimates of the winds at high-latitudes. (orig.)

Necrotizing enterocolitis, pathogenesis and the protector effect of prenatal corticosteroids Enterocolite necrosante: resposta imflamatória x corticoterapia pré-natal

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Alexander Roberto Precioso

2002-09-01

Full Text Available Necrotizing enterocolitis is the most frequently occurring gastrointestinal disorder in premature neonates. Animal models of necrotizing enterocolitis and prenatal administration of cortisone have demonstrated that cortisone may accelerate maturation of the mucosal barrier, therefore reducing the incidence of this gastrointestinal disorder. The authors present a review of the literature of the most important risk factors associated with necrotizing enterocolitis, such as inflammatory gastrointestinal mediators, enteral feeding and bacterial colonization, and immaturity of the gastrointestinal barrier, and we emphasize the necessity for additional studies to explore the prenatal administration of cortisone as a preventive strategy for necrotizing enterocolitis.A enterocolite necrosante é a mais freqüente patologia gastrointestinal adquirida no período neonatal, acometendo preferencialmente o recém-nascido prematuro. Estudos experimentais sugerem que a corticoterapia pré-natal acelera a maturação da mucosa gastrintestinal, levando a diminuição da incidência desta doença. Os autores apresentam uma revisão da literatura em relação aos principais fatores fisiopatológicos associados a enterocolite necrosante, tais como mediadores inflamatórios gastrintestinais, nutrição enteral e colonização bacteriana e imaturidade gastrintestinal e enfatizam a necessidade de mais estudos que avaliem a influencia da corticoterapia pré-natal com fator de prevenção da enterocolite necrosante.

Gene expression profiling in necrotizing enterocolitis reveals pathways common to those reported in Crohn's disease

NARCIS (Netherlands)

É. Tremblay (Éric); M.-P. Thibault (Marie-Pier); E. Ferretti (Emanuela); C. Babakissa (Corentin); V. Bertelle (Valérie); M. Bettolli (Marcos); K.M. Burghardt (Karolina Maria); J.-F. Colombani (Jean-François); D. Grynspan (David); E. Levy (Emile); P. Lu (Peng); S. Mayer (Sandeep); D. Ménard (Daniel); O. Mouterde (Olivier); I.B. Renes (Ingrid); E.G. Seidman (Ernest G.); J.-F. Beaulieu (Jean-François)

2016-01-01

textabstractBackground: Necrotizing enterocolitis (NEC) is the most frequent life-threatening gastrointestinal disease experienced by premature infants in neonatal intensive care units. The challenge for neonatologists is to detect early clinical manifestations of NEC. One strategy would be to

Nutritive support in short Bowel syndrome (sbs

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Simić Dušica

2003-01-01

Full Text Available Short bowel syndrome most commonly result after bowel resection for necrosis of the bowel. It may be caused by arterial or venous thrombosis, volvolus and in children, necrotizing enterocolitis. The other causes are Crohn,s disease intestinal atresia. The factors influencing the risk on short bowel syndrome are the remaining length of the small bowel, the age of onset, the length of the colon, the presence or absence of the ileo-coecal valve and the time after resection. Besides nutritional deficiencies there some other consequences of extensive resections of the small intestine (gastric acid hypersecretion, d-lactic acidosis, nephrolithiasis, cholelithiasis, which must be diagnosed, treated, and if possible, prevented. With current therapy most patients with short bowel have normal body mass index and good quality of life.

Probiotics and necrotizing enterocolitis.

Science.gov (United States)

Fleming, Paul; Hall, Nigel J; Eaton, Simon

2015-12-01

Probiotics for the prevention of necrotizing enterocolitis have attracted a huge interest. Combined data from heterogeneous randomised controlled trials suggest that probiotics may decrease the incidence of NEC. However, the individual studies use a variety of probiotic products, and the group at greatest risk of NEC, i.e., those with a birth weight of less than 1000 g, is relatively under-represented in these trials so we do not have adequate evidence of either efficacy or safety to recommend universal prophylactic administration of probiotics to premature infants. These problems have polarized neonatologists, with some taking the view that it is unethical not to universally administer probiotics to premature infants, whereas others regard the meta-analyses as flawed and that there is insufficient evidence to recommend routine probiotic administration. Another problem is that the mechanism by which probiotics might act is not clear, although some experimental evidence is starting to accumulate. This may allow development of surrogate endpoints of effectiveness, refinement of probiotic regimes, or even development of pharmacological agents that may act through the same mechanism. Hence, although routine probiotic administration is controversial, studies of probiotic effects may ultimately lead us to effective means to prevent this devastating disease.

Activation of iNKT Cells Prevents Salmonella-Enterocolitis and Salmonella-Induced Reactive Arthritis by Downregulating IL-17-Producing γδT Cells

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Mariángeles Noto Llana

2017-09-01

Full Text Available Reactive arthritis (ReA is an inflammatory condition of the joints that arises following an infection. Salmonella enterocolitis is one of the most common infections leading to ReA. Although the pathogenesis remains unclear, it is known that IL-17 plays a pivotal role in the development of ReA. IL-17-producers cells are mainly Th17, iNKT, and γδT lymphocytes. It is known that iNKT cells regulate the development of Th17 lineage. Whether iNKT cells also regulate γδT lymphocytes differentiation is unknown. We found that iNKT cells play a protective role in ReA. BALB/c Jα18−/− mice suffered a severe Salmonella enterocolitis, a 3.5-fold increase in IL-17 expression and aggravated inflammation of the synovial membrane. On the other hand, activation of iNKT cells with α-GalCer abrogated IL-17 response to Salmonella enterocolitis and prevented intestinal and joint tissue damage. Moreover, the anti-inflammatory effect of α-GalCer was related to a drop in the proportion of IL-17-producing γδT lymphocytes (IL17-γδTcells rather than to a decrease in Th17 cells. In summary, we here show that iNKT cells play a protective role against Salmonella-enterocolitis and Salmonella-induced ReA by downregulating IL17-γδTcells.

Preterm Birth and Necrotizing Enterocolitis Alter Gut Colonization in Pigs

DEFF Research Database (Denmark)

Cilieborg, Malene S.; Boye, Mette; Mølbak, Lars

2011-01-01

perfringens predisposes to NEC. By using terminal-RFLP and FISH, we characterized the gut microbiota of preterm, caesarean-delivered, formula-fed pigs (n = 44) with or without NEC and of formula- or colostrum-fed term, and vaginally born pigs (n = 13). A different microbiota with high C. perfringens abundance......Necrotizing enterocolitis (NEC) in preterm neonates is dependent on bacterial colonization, but it remains unclear whether a particular microbiota or specific pathogens are involved. We hypothesized that gut colonization differs between preterm and term neonates and that overgrowth of Clostridium...

Avanços em enterocolite necrosante Advances in necrotizing enterocolitis

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Nelson Diniz de Oliveira

2005-03-01

Full Text Available OBJETIVO: Avaliar relatos recentes sobre a enterocolite necrosante, com especial interesse na etiopatogenia, manejo e prevenção. FONTE DOS DADOS: Os artigos utilizados nessa revisão consistem em ensaios randomizados ou semi-randomizados, estudos de caso-controle, metanálises e artigos de revisão recentemente publicados. Alguns outros artigos foram selecionados devido à sua importância para o tema. RESULTADOS: A enterocolite necrosante é uma importante causa de morbimortalidade neonatal em prematuros. Entre esses, os nascidos com retardo de crescimento intra-uterino apresentam um risco mais elevado. O processo fisiopatológico inicia-se intra-útero e continua após o nascimento. Entre outros fatores envolvidos na fisiopatologia, estão a ação da arginina na produção do óxido nítrico intestinal e a ação do fator de crescimento epidérmico na regeneração celular. A perfuração intestinal ainda é um problema cirúrgico, e evidências melhores quanto à sua abordagem precisam ser avaliadas. Após a cirurgia, a extensão da alça intestinal remanescente, a preservação da válvula ileocecal, assim como a utilização precoce de leite materno ou solução de aminoácidos, são determinantes na duração da nutrição parenteral e no sucesso da readaptação intestinal. Estratégias preventivas estão centradas nas práticas alimentares e, recentemente, na suplementação de aminoácidos. CONCLUSÃO: Com um melhor entendimento do processo fisiopatológico, do manejo clínico e cirúrgico, assim como das medidas de prevenção, importantes resultados serão alcançados em termos de redução da morbimortalidade conseqüente à enterocolite necrosante.OBJECTIVE: To evaluate recently reported findings on necrotizing enterocolitis, Paying particular attention to pathogenesis, management and preventative strategies. DATA SOURCES: The articles covered in this report consist of randomized and quasi-randomized trials, case control studies

Fatal necrotising enterocolitis due to mydriatic eye drops.

Science.gov (United States)

Ozgun, Uygur; Demet, Terek; Ozge, Koroglu A; Zafer, Dokumcu; Murat, Sezak; Mehmet, Yalaz; Nilgun, Kultursay

2014-05-01

Retinopathy of prematurity (ROP) is a serious problem of preterm infants which may lead to impairment of vision and even to blindness if untreated. Routine eye examination is necessary for early diagnosis and treatment of ROP in preterm infants. Mydriatic eye drops (cyclopentolate, tropicamide and phenylephrine) are applied before the ophthalmic examination. These agents are rarely absorbed to systemic circulation and in some cases result with serious side effects like skin rash, tachycardia, feeding intolerance, discomfort, apnea, gastric dilatation and ileus, despite different treatment models and dosage reducing strategies. We report here a preterm patient who died because of severe diffuse necrotizing enterocolitis (NEC) after topical application of 0.5% cyclopentolate and 1.25% phenylephrine during ROP screening to emphasise the serious side effects of these agents.

Necrotizing enterocolitis (NEC: what’s going on

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Mario De Curtis

2013-06-01

Full Text Available Necrotizing enterocolitis (NEC is among the most common and devastating diseases in neonates and, despite the significant advances in neonatal care and clinical and basic science investigations, its etiology remains incompletely understood, specific treatment strategies are lacking, and morbidity and mortality from this disease remain high. Recent improvements in the pathophysiology of NEC may have therapeutic consequences. Toll-like receptors and intestinal microflora play an increasing role in the pathogenesis of NEC. Pharmacologic inhibition of TLR signaling, the use of novel nutritional strategies, and microflora modulation may represent novel promising approaches to the prevention and treatment of NEC. This review focuses on current and future therapeutic perspectives, starting from the recent acquisitions in the pathogenic mechanisms of NEC. Proceedings of the 9th International Workshop on Neonatology · Cagliari (Italy · October 23rd-26th, 2013 · Learned lessons, changing practice and cutting-edge research

Studies on five cases of radiation enterocolitis

Energy Technology Data Exchange (ETDEWEB)

Yasunaga, Akira; Shibata, Okihiko; Kubo, Hironobu; Tomonari, Kazuhide; Hadama, Tetsuo; Uchida, Yuzou; Shirabe, Joji (Oita Medical Coll. (Japan))

1990-05-01

Five patients with radiation enterocolitis who were surgically treated are reported. The 5 patients had received irradiation therapy more than 5,000 rad for ginecologic malignancies. The period for the onset of symptoms of irradiation enteritis ranged from 8 months to 20 years however, 3 of them developed the symptoms within one year after irradiation therapy. Emergency surgery was carried out for bowel obstruction in 3 cases and for intestinal perforation in 2 cases. Bowel resection and primary anastomosis were performed by a one-step approach in 4 of them. Transient colostomy as preventive measure was added in 2 cases. It is very important for the one-step approach to resect the intestine at the healthy part far from the lesion of irradiation enteritis and to add a transient colostomy to prevent anastomolic insufficiency. Furthermore, suture material should be used less tissue reactive one. (author).

Studies on five cases of radiation enterocolitis

International Nuclear Information System (INIS)

Yasunaga, Akira; Shibata, Okihiko; Kubo, Hironobu; Tomonari, Kazuhide; Hadama, Tetsuo; Uchida, Yuzou; Shirabe, Joji

1990-01-01

Five patients with radiation enterocolitis who were surgically treated are reported. The 5 patients had received irradiation therapy more than 5,000 rad for ginecologic malignancies. The period for the onset of symptoms of irradiation enteritis ranged from 8 months to 20 years however, 3 of them developed the symptoms within one year after irradiation therapy. Emergency surgery was carried out for bowel obstruction in 3 cases and for intestinal perforation in 2 cases. Bowel resection and primary anastomosis were performed by a one-step approach in 4 of them. Transient colostomy as preventive measure was added in 2 cases. It is very important for the one-step approach to resect the intestine at the healthy part far from the lesion of irradiation enteritis and to add a transient colostomy to prevent anastomolic insufficiency. Furthermore, suture material should be used less tissue reactive one. (author)

Clostridial Strain-Specific Characteristics Associated with Necrotizing Enterocolitis.

Science.gov (United States)

Schönherr-Hellec, Sophia; Klein, Geraldine L; Delannoy, Johanne; Ferraris, Laurent; Rozé, Jean Christophe; Butel, Marie José; Aires, Julio

2018-04-01

We aimed at identifying potential bacterial factors linking clostridia with necrotizing enterocolitis (NEC). We compared the phenotypic traits, stress responses, cellular cytotoxicity, and inflammatory capabilities of the largest collection of Clostridium butyricum and Clostridium neonatale strains isolated from fecal samples of NEC preterm neonates (PN) and control PNs. When strain characteristics were used as explanatory variables, a statistical discriminant analysis allowed the separation of NEC and control strains into separate groups. Strains isolated from NEC PN were characterized by a higher viability at 30°C ( P = 0.03) and higher aerotolerance ( P = 0.01), suggesting that NEC strains may have a competitive and/or survival advantage in the environmental gastrointestinal tract conditions of NEC PN. Heat-treated NEC bacteria induced higher production of interleukin-8 in Caco-2 cells ( P = 0.03), suggesting proinflammatory activity. In vitro , bacteria, bacterial components, and fecal filtrates showed variable cytotoxic effects affecting the cellular network and/or cell viability, without specific association with NEC or control samples. Altogether, our data support the existence of a specific clostridial strain signature associated with NEC. IMPORTANCE Clostridia are part of the commensal microbiota in preterm neonates (PN). However, microbiota analyses by culture and metagenomics have linked necrotizing enterocolitis (NEC) and intestinal colonization with clostridial species. Nevertheless, little is known about the specific characteristics that may be shared by clostridia associated with NEC compared to commensal clostridia. Therefore, our goal was to identify specific bacterial factors linking clostridial strains with NEC. We report the existence of a specific bacterial signature associated with NEC and propose that activation of the innate immune response may be a unifying causative mechanism for the development of NEC independent of a specific pathogenic

Cytomegalovirus-associated colitis mimicking necrotizing enterocolitis – A near miss diagnosis of neonatal colonic stricture

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Fanny Yeung

2014-10-01

Full Text Available Although cytomegalovirus (CMV is a common congenital infection in neonates, most patients are asymptomatic. Gastrointestinal manifestation is unusual. In this report, we described a newborn with perinatal CMV infection presented with symptoms mimicking necrotizing enterocolitis. We hope to alert clinicians about this possible diagnosis when managing newborn gastrointestinal diseases.

Enterocolic lymphocytic phlebitis: an unusual cause of abdominal complaints.

Science.gov (United States)

Gałązka, Krystyna; Tokarek, Tomasz; Gach, Tomasz; Szpor, Joanna

2012-03-01

Enterocolic lymphocytic phlebitis (ELP) is a rare disease of unknown etiology involving most often the intramural and mesenteric small and medium-sized veins of the gastrointestinal tract. The diagnosis of the disorder is based on the histopathological examination of a surgical specimen as endoscopically obtained diagnostic material is usually too superficial. Clinical manifestation of ELP most frequently is characterized by acute symptoms, such as acute abdomen, signs suggesting acute appendicitis, gastrointestinal hemorrhage, sometimes it manifests as chronic gastrointestinal complaints. We report, to our knowledge for the first time in Poland, a case of ELP with clinical symptoms pointing to acute appendicitis, on laparoscopy manifesting as a tumorous mass in the colonic wall with an unchanged appendix.

Investigation of the early intestinal microflora in premature infants with/without necrotizing enterocolitis using two different methods

DEFF Research Database (Denmark)

Smith, Birgitte; Bodé, Susan; Skov, Thomas

2012-01-01

The pathophysiology of necrotizing enterocolitis (NEC) is multifactorial, and gastrointestinal bacteria are thought to play an important role. In this study, the role of microflora in the gastrointestinal tract of neonates with NEC was assessed by comparing cases with controls....

The role of immunonutrients in the prevention of necrotizing enterocolitis in preterm very low birth weight infants

DEFF Research Database (Denmark)

Zhou, Ping; Li, Yanqi; Ma, Li-Ya

2015-01-01

Necrotizing enterocolitis (NEC) is a critical intestinal emergency condition, which mainly occurs in preterm very low birth weight (PVLBW) infants. Despite remarkable advances in the care of PVLBW infants, with considerable improvement of the survival rate in recent decades, the incidence of NEC...

Surgery for two cases with markedly impaired QOL(Quality of Life) of radiation enterocolitis

International Nuclear Information System (INIS)

Shiba, Tadaaki; Tanishima, Satoru; Sato, Masahiko; Yanagisawa, Terumasa; Yoshino, Masaaki; Takatsuka, Jun

1996-01-01

Two cases of radiation enterocolitis with markedly impaired QOL for a long period were reported. Case 1: A 61 years old female. She received 60 Gy irradiation post hysterectomy and ovariectomy due to uterine cancer at the age of 39 y. She suffered from fecal incontinence and anal pain at 58 y, was diagnosed to have radiation colitis. She was hospitalized due to neurosis, anal pain and hypertension at 59 y. She received nephrostomy due to hydronephrosis and ureterostenosis at 60 y, and colostomy and ileal conduit formation due to anal pain and dyschezia. Case 2: A 70 years old female who received 60 Gy irradiation post surgery similar to case 1 at the age of 61 y. She suffered from ileus, intestinal retention and hydronephrosis thereafter. She was hospitalized due to metastasis of the cancer to sacred bone, and received further 40 Gy radiation therapy at the age of 65 y. Up to the present, she received several operations such as ileostomy, nephrostomy and hemorrhoids excision. It is important for surgeons to understand the actual QOL of patients with radiation enterocolitis. (K.H.)

Surgery for two cases with markedly impaired QOL(Quality of Life) of radiation enterocolitis

Energy Technology Data Exchange (ETDEWEB)

Shiba, Tadaaki; Tanishima, Satoru; Sato, Masahiko; Yanagisawa, Terumasa; Yoshino, Masaaki; Takatsuka, Jun [Toho Univ., Tokyo (Japan). School of Medicine

1996-10-01

Two cases of radiation enterocolitis with markedly impaired QOL for a long period were reported. Case 1: A 61 years old female. She received 60 Gy irradiation post hysterectomy and ovariectomy due to uterine cancer at the age of 39 y. She suffered from fecal incontinence and anal pain at 58 y, was diagnosed to have radiation colitis. She was hospitalized due to neurosis, anal pain and hypertension at 59 y. She received nephrostomy due to hydronephrosis and ureterostenosis at 60 y, and colostomy and ileal conduit formation due to anal pain and dyschezia. Case 2: A 70 years old female who received 60 Gy irradiation post surgery similar to case 1 at the age of 61 y. She suffered from ileus, intestinal retention and hydronephrosis thereafter. She was hospitalized due to metastasis of the cancer to sacred bone, and received further 40 Gy radiation therapy at the age of 65 y. Up to the present, she received several operations such as ileostomy, nephrostomy and hemorrhoids excision. It is important for surgeons to understand the actual QOL of patients with radiation enterocolitis. (K.H.)

Antibiotic-Associated Apoptotic Enterocolitis in the Absence of a Defined Pathogen: The Role of Intestinal Microbiota Depletion*

Science.gov (United States)

Wurm, Philipp; Spindelboeck, Walter; Krause, Robert; Plank, Johannes; Fuchs, Gottfried; Bashir, Mina; Petritsch, Wolfgang; Halwachs, Bettina; Langner, Cord; Högenauer, Christoph

2017-01-01

Objective: Antibiotic therapy is a major risk factor for the development of diarrhea and colitis with varying severity. Often the origin of antibiotic-associated gastrointestinal deterioration remains elusive and no specific infectious agents could be discerned. Patients: We represent three cases of intractable high-volume diarrhea associated with combined antibiotic and steroid therapy in critically ill patients not fitting into established disease entities. Cases presented with severe apoptotic enterocolitis resembling acute intestinal graft-versus-host-disease. Microbiologic workup precluded known enteropathogens, but microbiota analysis revealed a severely depleted gut microbiota with concomitant opportunistic pathogen overgrowth. Interventions: Fecal microbiota transplantation, performed in one patient, was associated with correction of dysbiosis, rapid clinical improvement, and healing of enterocolitis. Conclusions: Our series represents a severe form of antibiotic-associated colitis in critically ill patients signified by microbiota depletion, and reestablishment of a physiologic gastrointestinal microbiota might be beneficial for this condition. PMID:28333760

Salmonella enterica serovar Enteritidis enterocolitis during late stages of gestation induces an adverse pregnancy outcome in the murine model.

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Mariángeles Noto Llana

Full Text Available Foodborne diseases caused by Salmonella enterica serovar Enteritidis (S. Enteritidis are a significant health problem. Pregnancy, state of immunological tolerance, is a predisposing condition for the development of infections with intracellular pathogens. Salmonella species can cause pregnancy complications such as chorioamnionitis, transplacental fetal infection, pre term labor, abortions, neonatal and maternal septicemia. However, the specific mechanisms by which Salmonella infections trigger these alterations are not clear. In the present work, using a self-limiting enterocolitis murine model, we show that the ingestion of a low dose of S. Enteritidis at late stages of pregnancy (day 15 of gestation is sufficient to induce massive maternal infection. We found that Salmonella infection leads to 40% of pre term delivery, 33% of abortion and fetal growth restriction. Placental dysfunction during S. Enteritidis enterocolitis was confirmed through cellular infiltration and hypoxia markers (MPO activity and COX-1 and COX-2 expression, respectively. Apoptosis in placental tissue due to Salmonella infection was also evident at day 18 of gestation when investigated by morphometric procedure, DNA fragmentation and Fas/FasL expression. Also, the expression of IFN-γ, TNF-α, IL-17 and IL-10 was up regulated in response to Salmonella not only in placenta, but also in amniotic fluid and maternal serum. Altogether, our results demonstrate that S. Enteritidis enterocolitis during late stages of gestation causes detrimental effect on pregnancy outcome.

Two Dual Ion Spectrometer Flight Units of the Fast Plasma Instrument Suite (FPI) for the Magnetospheric Multiscale Mission (MMS)

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Adams, Mitzi

2014-01-01

Two Dual Ion Spectrometer flight units of the Fast Plasma Instrument Suite (FPI) for the Magnetospheric Multiscale Mission (MMS) have returned to MSFC for flight testing. Anticipated to begin on June 30, tests will ensue in the Low Energy Electron and Ion Facility of the Heliophysics and Planetary Science Office (ZP13), managed by Dr. Victoria Coffey of the Natural Environments Branch of the Engineering Directorate (EV44). The MMS mission consists of four identical spacecraft, whose purpose is to study magnetic reconnection in the boundary regions of Earth's magnetosphere.

Yersinia enterocolitica bacteremia and enterocolitis in a previously healthy 20-month-old girl.

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Ito, Takao; Suzuki, Teruaki; Kawase, Jun; Fukushima, Hiroshi; Nanao, Kenji

2012-10-01

Yersinia enterocolitica is a gram-negative bacillus that can cause illness ranging from a self-limiting enterocolitis to life-threatening bacteremia. Y. enterocolitica biotype 1B, serotype O:8 (1B/O:8), is the most pathogenic of the Yersinia species because of the presence of the high-pathogenicity island and the Yersinia virulence plasmid (pYV). Here, we report a pediatric case of Y. enterocolitica 1B/O:8 bacteremia and enterocolitis. A 20-month-old girl was admitted to hospital with fever,pharyngitis, and abdominal pain on day 2. Blood culture on admission was positive for Y. enterocolitica 1B/O:8. Stool culture on day 5 after cefotaxime treatment was also positive for Y. enterocolitica 1B/O:8, but only after cold enrichment at 4°C for 3 weeks. PCR assays identified the pYV only in stool specimens, indicating that strains from routine blood culture at 37°C lacked the pYV. The present case showed the usefulness of stool culture with cold enrichment and agglutination test for the diagnosis of Y. enterocolitica infection. We would therefore like to emphasize the importance of collection and preservation of stool specimens for the identification of pYV. To our knowledge, this is the first reported pediatric case of Y. enterocolitica 1B/O:8 bacteremia.

Similar efficacy of human banked milk and bovine colostrum to decrease incidence of necrotizing enterocolitis in preterm piglets

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Jensen, Michael L.; Sangild, Per Torp; Lykke, Mikkel

2013-01-01

Preterm birth and formula feeding predispose to necrotizing enterocolitis (NEC) in infants. As mother's milk is often absent following preterm delivery, infant formula (IF) and human donor milk (HM) are frequently used as alternatives. We have previously shown that porcine and bovine colostrum (BC...

Probiotics to prevent necrotising enterocolitis in very preterm infants

DEFF Research Database (Denmark)

Lambæk, Irina Dobychina; Fonnest, Gert; Gormsen, Magdalena

2016-01-01

INTRODUCTION: Meta-analyses of randomised trials have shown that probiotics reduce the risk of necrotising enterocolitis (NEC) in preterm infants. However, the generalisability of these results, particularly for the most preterm infants, remains unresolved. Hence, we wanted to evaluate the benefit...... of implementing prophylactic use of probiotics as standard care in infants younger than 30 weeks of gestation. METHODS: Two three-year periods were compared. The first period was prior to a policy change. In this period no probiotics were used. The second period featured routine administration of probiotics...... period (median six versus 14 days, p = 0.004). No side effects and no blood cultures with lactobacillus or bifidobacterium were observed. CONCLUSIONS: This historically controlled study did not indicate that probiotics had a significant effect on NEC. We continue our practice, but larger cohort studies...

The pathophysiology of necrotizing enterocolitis in preterm infants : New insights in the interaction between the gut and its microbiota

NARCIS (Netherlands)

Heida, Fardou Hadewych

2016-01-01

Necrotizing enterocolitis (NEC) is a severe gastrointestinal disorder affecting the preterm infant. The underlying cause of NEC is partly unknown. This thesis studied the gut flora, the intestinal barrier function, and the intestinal bloodcirculation contributing to NEC. We observed NEC-associated

Severe neurodevelopmental disability and healthcare needs among survivors of medical and surgical necrotizing enterocolitis: A prospective cohort study.

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Fullerton, Brenna S; Hong, Charles R; Velazco, Cristine S; Mercier, Charles E; Morrow, Kate A; Edwards, Erika M; Ferrelli, Karla R; Soll, Roger F; Modi, Biren P; Horbar, Jeffrey D; Jaksic, Tom

2017-10-12

This study characterizes neurodevelopmental outcomes and healthcare needs of extremely low birth weight (ELBW) survivors of necrotizing enterocolitis (NEC) compared to ELBW infants without NEC. Data were collected prospectively on neonates born 22-27weeks' gestation or 401-1000g at 47 Vermont Oxford Network member centers from 1999 to 2012. Detailed neurodevelopmental evaluations were conducted at 18-24months corrected age. Information regarding rehospitalizations, postdischarge surgeries, and feeding was also collected. "Severe neurodevelopmental disability" was defined as: bilateral blindness, hearing impairment requiring amplification, inability to walk 10 steps with support, cerebral palsy, and/or Bayley Mental or Psychomotor Developmental Index neurodevelopmental disability, nearly half underwent postdischarge operations, and a quarter required tube feeding at home. At 18-24months, extremely low birth weight survivors of necrotizing enterocolitis were at markedly increased risk (pneurodevelopmental disability, postdischarge surgery, and tube feeding. II (prospective cohort study with <80% follow-up rate). Copyright © 2017 Elsevier Inc. All rights reserved.

Noninvasive measurement of fecal calprotectin and serum amyloid A combined with intestinal fatty acid-binding protein in necrotizing enterocolitis.

NARCIS (Netherlands)

Reisinger, K.W.; Zee, D.C. van der; Brouwers, H.A.A.; Kramer, B.W.; Heurn, L.W.E. van; Buurman, W.A.; Derikx, J.P.

2012-01-01

BACKGROUND: Diagnosis of necrotizing enterocolitis (NEC), prevalent in premature infants, remains challenging. Enterocyte damage in NEC can be assessed by intestinal fatty acid-binding protein (I-FABP), with a sensitivity of 93% and a specificity of 90%. Numerous markers of inflammation are known,

The Incidence of Necrotizing Enterocolitis Is Increased Following Probiotic Administration to Preterm Pigs

DEFF Research Database (Denmark)

Cilieborg, Malene Skovsted; Thymann, Thomas; Siggers, Richard

2011-01-01

Preterm birth and necrotizing enterocolitis (NEC) is associated with inappropriate gut colonization and immunity, which may be improved by probiotic bacteria. Using a preterm pig model of NEC, we investigated the effects of probiotics on intestinal structure, function, microbiology, and immunology...... with controls (n = 14). All pigs received parenteral nutrition for 2 d followed by enteral formula feeding until tissue collection on d 5. Compared with control pigs, intestinal weight was lower and NEC incidence was higher in both groups given probiotics (64–67 vs. 14%; P...

Pasteurization of breastmilk decreases the rate of postnatally acquired cytomegalovirus infections, but shows a nonsignificant trend to an increased rate of necrotizing enterocolitis in very preterm infants--a preliminary study.

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Stock, Katharina; Griesmaier, Elke; Brunner, Barbara; Neubauer, Vera; Kiechl-Kohlendorfer, Ursula; Trawöger, Rudolf

2015-03-01

This study assessed whether feeding preterm infants unpasteurized breastmilk (1) decreases the rate of late-onset sepsis and necrotizing enterocolitis and (2) increases the rate of postnatally acquired cytomegalovirus infections. Between January 2008 and July 2013, preterm infants below 32 completed weeks of gestational age admitted to the neonatal intensive care unit of Innsbruck Medical University (Innsbruck, Austria) (n=344) were eligible for the study. Of those, 323 fed breastmilk were retrospectively enrolled in the study. Two groups were formed, with 164 infants being fed unpasteurized and 159 infants being fed pasteurized breastmilk. There was no significant difference in the rate of late-onset sepsis or necrotizing enterocolitis between the unpasteurized and pasteurized breastmilk groups (late-onset sepsis, 15.9% versus 15.1% [p=0.486]; necrotizing enterocolitis, 2.4% versus 4.4% [p=0.254]). The number of infants diagnosed with postnatally acquired cytomegalovirus infection was significantly higher in the unpasteurized group (39.3%) compared with the pasteurized group (4.2%) (p=0.008). Feeding preterm infants unpasteurized breastmilk increases the rate of postnatally acquired cytomegalovirus infections. However, we also demonstrate a nonsignificant trend to a decreased rate of necrotizing enterocolitis in the unpasteurized group, which needs to be confirmed in larger studies.

Gene expression profiling in necrotizing enterocolitis reveals pathways common to those reported in Crohn's disease

OpenAIRE

Tremblay, Éric; Thibault, Marie-Pier; Ferretti, Emanuela; Babakissa, Corentin; Bertelle, Valérie; Bettolli, Marcos; Burghardt, Karolina Maria; Colombani, Jean-François; Grynspan, David; Levy, Emile; Lu, Peng; Mayer, Sandeep; Ménard, Daniel; Mouterde, Olivier; Renes, Ingrid

2016-01-01

textabstractBackground: Necrotizing enterocolitis (NEC) is the most frequent life-threatening gastrointestinal disease experienced by premature infants in neonatal intensive care units. The challenge for neonatologists is to detect early clinical manifestations of NEC. One strategy would be to identify specific markers that could be used as early diagnostic tools to identify preterm infants most at risk of developing NEC or in the event of a diagnostic dilemma of suspected disease. As a first...

Inventário de Personalidade de Freiburg-Revisto (FPI-R: estudo de validação junto de amostra de estudantes universitários

Directory of Open Access Journals (Sweden)

Isabel Soares

2005-01-01

Full Text Available En este estudio instrumental se presentan los primeros datos de validación de la versión portuguesa del Inventario de Personalidad de Freiburg- Revisado (FPI-R en una muestra de 1099 estudiantes universitarios. El FPI-R está compuesto por 138 ítems de respuesta dicotómica (verdadero/falso distribuidos en 12 escalas: 10 escalas básicas (Satisfacción con la vida, Orientación social, Orientación hacia el desempeño, Inhibición, Irritabilidad, Agresividad, Solicitud, Quejas somáticas, Preocupopaciones por la salud y Sinceridad y 2 escalas suplementarias (Extraversión y Emocionalidad. Este cuestionario trata de evaluar constructos relativamente amplios que contienen subconstructos delimitados, estando organizados en escalas unipolares (por ejemplo, pocas quejas somáticas - muchas quejas somáticas y bipolares (por ejemplo, insatisfacción con la vida – satisfaccción con la vida. Los resultados revelan que, a pesar de que el instrumento presenta una estructura factorial semejante a la original, se encontraron algunas debilidades en terminos de fiabilidad de las subescalas. Se discuten los resultados obtenidos, las limitaciones encontradas, proponiéndose posibles soluciones a desarrollar en futuros estudios.

Factores de riesgo asociados a la mortalidad por enterocolitis necrotizante

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Vivian R. Mena Miranda

1998-12-01

Full Text Available La enterocolitis necrotizante (ECN es una urgencia gastrointestinal de causa multifactorial muy relacionada con el neonato pretérmino. Su elevada mortalidad radica en la falta de prevención por el médico a cualquier nivel de atención y a su diagnóstico tardío en los grupos de riesgo. Se realizó un estudio retrospectivo de los 63 pacientes fallecidos por ECN durante un período de 25 años en el Hospital Pediátrico Docente "Centro Habana", donde se encontró que 71,4 % de los afectados eran de la raza blanca y el 68,2 % del sexo masculino. La edad más frecuente se halló en los menores de 3 meses de edad (36,5 % y el 46 % del total de la muestra estudiada tuvo un peso al nacer inferior a los 1 500 g. La prematuridad apareció asociada en el 55,5 % de los fallecidos y el 65 % tuvo lactancia mixta desde el momento de nacimiento.Necrotizing enterocolitis (NEC is a multifactorial caused gastrointestinal urgency closely related to the preterm newborn. Its high mortality has roots in the lack of prevention by the physician at any level of care, and due to a late diagnosis in risk groups. A retrospective study was carried out about 63 deceased patients as a result of NEC, along a 25 years period at the Centro Habana Teaching Pediatric Hospital, and it was found that 71,4 % of the affected infants were white, and that 68,2 % were masculin. The most frequent age was found in infants less than 3 months old (36,5 %, and 46 % of the total sample investigated had a birth weight lower than 1 500 g. Prematurity appeared associated in 55,5 % of the deceased, and 65 % had mixed lactation from the birth on.

Enterocolic lymphocytic phlebitis: clinicopathologic features and review of the literature.

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Ngo, Nyethane; Chang, Fuju

2007-07-01

Enterocolic lymphocytic phlebitis (ELP) is a recently described entity and is of unknown etiology and pathogenesis. It is characterized by phlebitis of the bowel wall and mesentery, without arterial involvement or evidence of systemic vasculitis. The clinical presentation of ELP is varied, but it most commonly manifests with signs of an acute abdomen. Clinical, radiologic, and endoscopic findings are often conflicting and misdiagnosis is common as venous thrombosis is not suspected. The diagnosis of ELP is obtained histologically. There is a spectrum of histologic features associated with ELP, which includes lymphocytic phlebitis, necrotizing phlebitis, granulomatous phlebitis, and myointimal hyperplasia. Other features include venous thrombi and acute ischemic changes of the intestine. Surgical resection of the affected bowel is usually curative and recurrences are rare. The clinical and histopathologic features of ELP are reviewed.

Intestinal fatty acid-binding protein levels in Necrotizing Enterocolitis correlate with extent of necrotic bowel: results from a multicenter study

NARCIS (Netherlands)

Heida, F.H.; Hulscher, J.B.; Schurink, M.; Timmer, A.; Kooi, E.M.; Bos, A.F; Bruggink, J.L.; Kasper, D.C.; Pones, M.; Benkoe, T.

2015-01-01

BACKGROUND: Intestinal fatty acid-binding protein (I-FABP) is considered as a specific marker for enterocyte damage in necrotizing enterocolitis (NEC). OBJECTIVE: The purpose of this study was to evaluate the association of plasma and urinary I-FABP levels with the extent of macroscopic intestinal

Is Xanthine oxidase activity in polycystic ovary syndrome associated with inflammatory and cardiovascular risk factors?

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Isık, Hatice; Aynıoglu, Oner; Tımur, Hakan; Sahbaz, Ahmet; Harma, Muge; Can, Murat; Guven, Berrak; Alptekin, Husnu; Kokturk, Furuzan

2016-08-01

The aim of this study is to examine women with polycystic ovary syndrome (PCOS) to determine the relationship between xanthine oxidase (XO) and oxidative stress, inflammatory status, and various clinical and biochemical parameters. In this cross-sectional study a total of 83 women including 45 PCOS patients and 38 healthy women were enrolled. We collected blood samples for XO and superoxide dismutase (SOD) activity, hormone levels, cholesterol values, and inflammatory markers. Body mass index (BMI) , waist-to-hip ratio (WHR), and blood pressure were assessed. Blood samples were taken for hormonal levels, cholesterol levels, fasting plasma glucose (FPG), fasting plasma insulin (FPI), homeostatic model assessment-insulin resistance (HOMA-IR) index, quantitative insulin sensitivity check index (QUICKI), C-reactive protein (CRP), white blood cell and neutrophil counts, XO and SOD activities. The basal hormone levels, triglyceride (TG) levels, TG/HDL-C (high density lipoprotein-cholesterol) ratios FPG, FPI and HOMA-IR levels were higher in PCOS patients compared to controls (pPCOS patients (pPCOS patients. Positive correlations between XO and inflammatory markers and cardiovascular disease risk factors suggest that XO plays an important role in the pathogenesis of PCOS and its metabolic complications. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Short Bowel Syndrome, a Case of Intestinal Rehabilitation

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Dianna Ramírez Prada

2015-05-01

Full Text Available Case: The objective is to present the successful experience of multidisciplinary management of a patient with short bowel syndrome and intestinal failure with progression to intestinal adaptation. This is a newly born premature with intestinal atresia type IV with multiple intestinal atresia who evolved to intestinal failure and required managed with prolonged parenteral nutritional support, multiple antibiotic schemes, prebiotics, multivitamins, enteral nutrition with elemental formula to achieve their adaptation intestinal until lead to a normal diet. The evolution of these patients intestinal failure is a challenge for the health team, as it not only involves the surgical management of your condition if not basic nutritional support, fluid and electrolyte balance, hepatic dysfunction cholestasis associated infections etc. Discussion: Short bowel syndrome with progression to intestinal failure in children is a condition whose prevalence is increasing worldwide, thanks to advances in neonatal intensive care, neonatal surgery, and nutritional support of patients with conditions such as gastroschisis, omphalocele and necrotizing enterocolitis. Despite the limitations of our health system, it is possible to offer a multidisciplinary and integrated to lead to intestinal adaptation treatment.

Pathophyisiology and risk factors of necrotizing enterocolitis in newborns less than 1.500g Fisiopatología y factores de riesgo para el desarrollo de enterocolitis necrosante en neonatos menores de 1.500 g

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Carolina Tamayo Múnera

2006-04-01

Full Text Available The Necrotizing enterocolitis (NEC is a serious illness of the newborn that affects mainly the preterm infant. It corresponds to a diffuse intestinal necrosis with more affection of the ileocecal region. The pathophysiology is complex and multiple risk factors can cause the illness in a susceptible infant. Among the different theories about their appearance is observed that prematurity, intestinal isquemia, bacterial colonization and enteral feeding are the main risk factors, which can be favored by prenatal, perinatal or postnatal conditions. The aim of this article is to review the pathophysiology and the risk factors described in the current evidence. La enterocolitis necrosante (ECN es una grave enfermedad propia del recién nacido (RN que afecta principalmente a los RN pretérmino (RNPT. Corresponde a una necrosis isquémica de la mucosa intestinal con mayor afección de la región ileocecal. La fisiopatología es compleja y en ella participan múltiples factores de riesgo que pueden ocasionar la enfermedad en un RN susceptible. Entre las diferentes teorías acerca de su aparición se observa reiteradamente que la prematuridad, la isquemia intestinal, la colonización bacteriana y la alimentación enteral se repiten como los factores de riesgo principales, los cuales pueden ser favorecidos por condiciones prenatales, perinatales o posnatales. Se revisan la fisiopatología y los factores de riesgo implicados en la génesis de esta enfermedad.

Maternal Risk Factors for Neonatal Necrotizing Enterocolitis

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March, Melissa I.; Gupta, Munish; Modest, Anna M.; Wu, Lily; Hacker, Michele R.; Martin, Camilia R.; Rana, Sarosh

2015-01-01

Objective This study aimed to investigate the relationship between maternal hypertensive disease and other risk factors and the neonatal development of necrotizing enterocolitis (NEC). Methods This was a retrospective case control study of infants with NEC from 2008 to 2012. The primary exposure of interest was maternal hypertensive disease, which has been hypothesized to put infants at risk for NEC. Other variables collected included demographics, pregnancy complications, medications, and neonatal hospital course. Data was abstracted from medical records. Results 28 cases of singleton neonates with NEC and 81 matched controls were identified and analyzed. There was no significant difference in the primary outcome. Fetuses with an antenatal diagnosis of growth restriction were more likely to develop NEC (p=0.008). Infants with NEC had lower median birth weight than infants without NEC (p=0.009). Infants with NEC had more late-onset sepsis (p=0.01) and mortality before discharge (p=0.001). Conclusions The factors identified by this case-control study that increased the risk of neonatal NEC included intrauterine growth restriction and lower neonatal birth weight. The primary exposure, hypertensive disease, did not show a significantly increased risk of neonatal NEC, however there was a nearly two-fold difference observed. Our study was underpowered to detect the observed difference. PMID:25162307

Clinicopathological features and managements of radiation enterocolitis

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Takashima, Shigeki; Kinami, Yoshio [Kanazawa Medical Univ., Ishikawa (Japan)

1990-11-01

During the past 12 years, delayed radiation enterocolitis developed in 22 patients treated for uterine cervical cancer or endometrial cancer. Their ages ranged from 36 to 77 years with an average age of 62. Ten patients received external irradiation and the other 12 patients received the combined external and internal irradiation. Radiation doses were 50 Gy-156 Gy with an average dose of 80 Gy. All patients had early radiation effects manifested by myxoid bloody stool (82%), diarrhea (46%), defecation disorder (18%), and tenesmus like symptoms (9%). These symptoms resolved within one month after irradiation. The most common symptom for delayed radiation effects was anal hemorrhage and melena (73%), followed by ileus like symptoms and abdominal pain (14%). Defecation disorder, diarrhea, and anemia were observed in one each patient (5%). Latency period ranged from 4 months to 13 years after radiation treatment, with the greatest number (86%) having it within one year and 3 months. There was no definitive correlation between radiation doses and latency period. Histopathological features included marked proliferation extending from the submucosal layer to the muscular layer, endometrial thickening of the small vessels, and thrombopoiesis. Although surgical treatment was performed in 20 patients, favorable outcome was not achieved. (N.K.).

Gene expression profiling in necrotizing enterocolitis reveals pathways common to those reported in Crohn?s disease

OpenAIRE

Tremblay, ?ric; Thibault, Marie-Pier; Ferretti, Emanuela; Babakissa, Corentin; Bertelle, Val?rie; Bettolli, Marcos; Burghardt, Karolina Maria; Colombani, Jean-Fran?ois; Grynspan, David; Levy, Emile; Lu, Peng; Mayer, Sandeep; M?nard, Daniel; Mouterde, Olivier; Renes, Ingrid B.

2016-01-01

Background Necrotizing enterocolitis (NEC) is the most frequent life-threatening gastrointestinal disease experienced by premature infants in neonatal intensive care units. The challenge for neonatologists is to detect early clinical manifestations of NEC. One strategy would be to identify specific markers that could be used as early diagnostic tools to identify preterm infants most at risk of developing NEC or in the event of a diagnostic dilemma of suspected disease. As a first step in this...

Enteral feeding induces diet-dependent mucosal dysfunction, bacterial proliferation, and necrotizing enterocolitis in preterm pigs on parenteral nutrition

DEFF Research Database (Denmark)

Bjørnvad, Charlotte R.; Thymann, Thomas; Deutz, Nicolaas E.

2008-01-01

Preterm neonates have an immature gut and metabolism and may benefit from total parenteral nutrition (TPN) before enteral food is introduced. Conversely,delayed enteral feeding may inhibit gut maturation and sensitize to necrotizing enterocolitis (NEC). Intestinal mass and NEC lesions were first...... formula after TPN. Conversely, colostrum milk diets improve gut maturation and NEC resistance in preterm pigs subjected to a few days of TPN after birth....

Impact of antibiotics on necrotizing enterocolitis and antibiotic-associated diarrhea

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Silverman, Michael A.; Konnikova, Liza; Gerber, Jeffrey S.

2017-01-01

Summary Antibiotics induce changes or dysbiosis of the intestinal microbiome. These antibiotic-induce changes may contribute to the pathogenesis of necrotizing enterocolitis (NEC) and antibiotic-associated diarrhea (AAD). Studies are beginning to unravel the contribution of specific groups of microbes to these diseases—most notably Gammaproteobacteria for NEC and bile acid- and carbohydrate-metabolizing microbes for AAD. Antibiotic-associated diarrhea occurs when antibiotic treatment induces diarrhea by altering the metabolic function of the patient’s intestinal microbiota leading to either an osmotic or infectious diarrhea, most notably Clostridium difficile infection (CDI). Antibiotic therapy impairs the host microbiota’s ability to resist colonization or expansion of pathogenic bacteria. In the case of CDI, there is growing evidence that microbiota-mediated bile acid metabolism is critical in the pathogenesis of this infection. Probiotics or other microbiota-targeted therapies may provide effective strategies to prevent and treat NEC and AAD. PMID:28164853

Non-IgE-mediated food allergies | Terblanche | South African ...

African Journals Online (AJOL)

mediated conditions such as atopic dermatitis and eosinophilic oesophagitis, and pure T-cell-mediated conditions such as food protein-induced enterocolitis syndrome, allergic proctocolitis and enteropathy syndromes. Diagnosing mixed or ...

Effects of canrenone in patients with metabolic syndrome.

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Derosa, Giuseppe; Bonaventura, Aldo; Bianchi, Lucio; Romano, Davide; D'Angelo, Angela; Fogari, Elena; Maffioli, Pamela

2013-11-01

Metabolic syndrome is becoming a common disease due to a rise in obesity rates among adults. The aim was to evaluate the effects of canrenone compared to placebo on metabolic and inflammatory parameters in patients affected by metabolic syndrome. A total of 145 patients were treated with placebo or canrenone, 50 mg/day, for 3 months and then 50 mg b.i.d. till the end of the study. Blood pressure, body weight, body mass index, fasting plasma glucose (FPG), fasting plasma insulin, HOMA-IR, lipid profile, plasma aldosterone, brain natriuretic peptide, high-sensitivity C-reactive protein (Hs-CRP), tumor necrosis factor-α (TNF-α) and M value were evaluated. A decrease of blood pressure was observed in canrenone group compared to baseline; moreover, systolic blood pressure value recorded after 6 months of canrenone therapy was lower than the one recorded with placebo. Canrenone gave a significant decrease of FPI and HOMA index, and an increase of M value both compared to baseline and to placebo. Canrenone also decreased triglycerides and FPG was not observed with placebo. Canrenone also decreased plasma aldosterone, Hs-CRP and TNF-α compared to baseline and to placebo. Canrenone seems to be effective in reducing some factors involved in metabolic syndrome and in improving insulin-resistance and the inflammatory state observed in these patients.

Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation.

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Manuri, Lucia; Morelli, Stefano; Agati, Salvatore; Saitta, Michele B; Oreto, Lilia; Mandraffino, Giuseppe; Iannace, Enrico; Iorio, Fiore S; Guccione, Paolo

2017-01-01

The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure. We reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol. The median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1-70 days), and the median hospital length of stay was 16 days (6-70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events. Our experience indicates that the combination of an "early hybrid approach", systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis.

Primary anastomosis or ostomy in necrotizing enterocolitis?

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Haricharan, Ramanathapura N; Gallimore, Jade Palazzola; Nasr, Ahmed

2017-11-01

In neonates requiring operation for necrotizing enterocolitis (NEC), the complications due to enterostomy (ES) and the need for another operation to restore continuity have prompted several surgeons to employ primary anastomosis (PA) after resection as the operative strategy of choice. Our objective was to compare primary anastomosis to stoma formation in this population using systematic review and meta-analysis. Publications describing both interventions were identified by searching multiple databases. Appropriate studies that reported outcomes after PA and ES for NEC were included for analysis that was performed using the MedCalc3000 software. Results are reported as odds ratios (OR, 95% CI). No randomized trials were identified. Twelve studies were included for the final analysis. Neonates who underwent PA were associated with significantly less risk of mortality when compared to those who underwent ES (OR 0.34, 95% CI 0.17-0.68, p 0.002), possibly due to differences in severity of NEC. Although the types of complications in these groups were different, there was no significant difference in risk of complication (OR 0.86, 0.55-1.33, p 0.50). In neonates undergoing an operation for severe NEC, there is no significant difference in the risk of complications between primary anastomosis and enterostomy. A definitive suggestion cannot be made regarding the choice of one operative strategy over another.

There is an association between disease location and gestational age at birth in newborns submitted to surgery due to necrotizing enterocolitis.

Science.gov (United States)

Feldens, Letícia; Souza, João C K de; Fraga, José C

2017-08-30

To evaluate if there are differences regarding disease location and mortality of necrotizing enterocolitis, according to the gestational age at birth, in newborns submitted to surgery due to enterocolite. A historical cohort study of 198 newborns submitted to surgery due to necrotizing enterecolitis in a tertiary hospital, from November 1991 to December 2012. The newborns were divided into different categories according to gestational age (<30 weeks, 30-33 weeks and 6 days, 34-36 weeks and 6 days, and ≥37 weeks), and were followed for 60 days after surgery. The inclusion criterion was the presence of histological findings of necrotizing enterocolitis in the pathology. Patients with single intestinal perforation were excluded. The jejunum was the most commonly affected site in extremely premature infants (p=0.01), whereas the ileum was the most commonly affected site in premature infants (p=0.002), and the colon in infants born at term (p<0.001). With the increasing gestational age, it was observed that intestinal involvement decreased for the ileum and the jejunum (decreasing from 45% to 0% and from 5% to 0%, respectively), with a progressive increase in colon involvement (0% to 84%). Total mortality rate was 45.5%, and no statistical difference was observed in the mortality at different gestational ages (p=0.287). In newborns submitted to surgery due to necrotizing enterocolitis, the disease in extremely preterm infants was more common in the jejunum, whereas in preterm infants, the most affected site was the ileum, and in newborns born close to term, it was the colon. No difference in mortality was observed according to the gestational age at birth. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Bovine colostrum is superior to enriched formulas in stimulating intestinal function and necrotising enterocolitis resistance in preterm pigs

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Møller, Hanne Kristine; Thymann, Thomas; Fink, Lisbeth Nielsen

2011-01-01

Milk contains immunomodulatory compounds that may be important to protect the immature intestine in preterm neonates from harmful inflammatory reactions involved in disorders like necrotising enterocolitis (NEC). We hypothesised that bovine colostrum and milk formulas enriched with sialic acids (SL......-enriched formula (n 13), while Expt 2 compared a control formula with bovine colostrum or formulas enriched with Gang or SL (n 4-6). OPN enrichment decreased NEC severity relative to control formula (P...

Application of Mitomycin C after dilation of an anastomotic stricture in a newborn with necrotizing enterocolitis

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Jonathan Green

2016-01-01

Full Text Available Necrotizing enterocolitis (NEC is a common life-threatening condition in premature infants. Bacterial translocation, localized inflammation and subsequent perforation often require surgery for source control and definitive treatment. Small and large intestinal strictures may result from either creation of a surgical anastomosis or the disease process itself. Current methods to treat strictures include, balloon dilation and surgical resection with or without anastomosis. We report the diagnosis and surgical management of a premature infant treated for NEC, who developed an anastomotic stricture and was successfully treated with topical Mitomycin C after balloon stricturoplasty.

Parenteral lipids and partial enteral nutrition affect hepatic lipid composition but have limited short term effects on formula-induced necrotizing enterocolitis in preterm piglets

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Vegge, Andreas; Thymann, Thomas; Lauritzen, Lotte

2015-01-01

Rapid transition from total parenteral nutrition (TPN) to enteral feeding is a risk factor for necrotizing enterocolitis (NEC) in preterm infants. We hypothesized that partial enteral nutrition with colostrum, increased proportion of n-3 polyunsaturated fatty acids (PUFA), or exclusion of lipid...

Neutropenic enterocolitis affecting the transverse colon: an unusual complication of chemotherapy.

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Ramsingh, Jason; Bolln, Carsten; Hodnett, Robert; Al-Ani, Ahmed

2014-05-02

A 66-year-old woman presented with a 1-day history of sudden onset of generalised abdominal pain associated with fever and vomiting. She was previously diagnosed with left breast cancer 2 months ago and completed a course of chemotherapy 1 week prior to presentation. She was clinically unwell with generalised tenderness in her abdomen. Blood investigations showed severe neutropenia. A CT scan was requested which reported a marked oedematous swelling of the transverse colon with features suggestive of a contained perforation. The decision was made to operate. Intraoperatively, the transverse colon was found to be thickened with omentum adherent focally around the distal third. A right hemicolectomy was performed with an end ileostomy and mucus fistula. The patient made a successful recovery and was discharged within 7 days of presenting. Pathology reported typical features of neutropenic enterocolitis affecting the transverse colon with a normal terminal ileum, caecum and ascending colon.

Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not.

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Haricharan, Ramanath N; Seo, Jeong-Meen; Kelly, David R; Mroczek-Musulman, Elizabeth C; Aprahamian, Charles J; Morgan, Traci L; Georgeson, Keith E; Harmon, Carroll M; Saito, Jacqueline M; Barnhart, Douglas C

2008-06-01

This study was conducted to determine the effect of age at diagnosis and length of ganglionated bowel resected on postoperative Hirschsprung-associated enterocolitis (HAEC). Children who underwent endorectal pull-through (ERPT) between January 1993 and December 2004 were retrospectively reviewed. t Test, analysis of variance, Kaplan-Meier, and Cox's proportional hazards analyses were performed. Fifty-two children with Hirschsprung disease (median age, 25 days; range, 2 days-16 years) were included. Nineteen (37%) had admissions for HAEC. Proportional hazards regression showed that HAEC admissions decreased by 30% with each doubling of age at diagnosis (P = .03) and increased 9-fold when postoperative stricture was present (P 5 cm]). No significant difference in the number of HAEC admissions during initial 2 years post-ERPT was seen between groups A (n = 18) and B (n = 18). The study had a power of 0.8 to detect a difference of 1 admission over 2 years. Children diagnosed with Hirschsprung disease at younger ages are at a greater risk for postoperative enterocolitis. Excising a longer margin of ganglionated bowel (>5 cm) does not seem to be beneficial in decreasing HAEC admissions.

Elevated levels of circulating cell-free DNA and neutrophil proteins are associated with neonatal sepsis and necrotizing enterocolitis in immature mice, pigs and infants

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Nguyen, Duc Ninh; Stensballe, Allan; Lai, Jacqueline C.Y.

2017-01-01

Preterm infants are highly susceptible to late-onset sepsis (LOS) and necrotizing enterocolitis (NEC), but disease pathogenesis and specific diagnostic markers are lacking. Circulating cell-free DNA (cfDNA) and immune cell-derived proteins are involved in multiple immune diseases in adults but ha...

Evaluation of fasting plasma insulin concentration as an estimate of insulin action in nondiabetic individuals: comparison with the homeostasis model assessment of insulin resistance (HOMA-IR).

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Abbasi, Fahim; Okeke, QueenDenise; Reaven, Gerald M

2014-04-01

Insulin-mediated glucose disposal varies severalfold in apparently healthy individuals, and approximately one-third of the most insulin resistant of these individuals is at increased risk to develop various adverse clinical syndromes. Since direct measurements of insulin sensitivity are not practical in a clinical setting, several surrogate estimates of insulin action have been proposed, including fasting plasma insulin (FPI) concentration and the homeostasis model assessment of insulin resistance (HOMA-IR) calculated by a formula employing fasting plasma glucose (FPG) and FPI concentrations. The objective of this study was to compare FPI as an estimate of insulin-mediated glucose disposal with values generated by HOMA-IR in 758 apparently healthy nondiabetic individuals. Measurements were made of FPG, FPI, triglyceride (TG), and high-density lipoprotein cholesterol (HDL-C) concentrations, and insulin-mediated glucose uptake was quantified by determining steady-state plasma glucose (SSPG) concentration during the insulin suppression test. FPI and HOMA-IR were highly correlated (r = 0.98, P HOMA-IR (r = 0.64). Furthermore, the relationship between FPI and TG (r = 0.35) and HDL-C (r = -0.40) was comparable to that between HOMA-IR and TG (r = 0.39) and HDL-C (r = -0.41). In conclusion, FPI and HOMA-IR are highly correlated in nondiabetic individuals, with each estimate accounting for ~40% of the variability (variance) in a direct measure of insulin-mediated glucose disposal. Calculation of HOMA-IR does not provide a better surrogate estimate of insulin action, or of its associated dyslipidemia, than measurement of FPI.

Factores de riesgo asociados a la mortalidad por enterocolitis necrotizante

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Vivian R. Mena Miranda

Full Text Available La enterocolitis necrotizante (ECN es una urgencia gastrointestinal de causa multifactorial muy relacionada con el neonato pretérmino. Su elevada mortalidad radica en la falta de prevención por el médico a cualquier nivel de atención y a su diagnóstico tardío en los grupos de riesgo. Se realizó un estudio retrospectivo de los 63 pacientes fallecidos por ECN durante un período de 25 años en el Hospital Pediátrico Docente "Centro Habana", donde se encontró que 71,4 % de los afectados eran de la raza blanca y el 68,2 % del sexo masculino. La edad más frecuente se halló en los menores de 3 meses de edad (36,5 % y el 46 % del total de la muestra estudiada tuvo un peso al nacer inferior a los 1 500 g. La prematuridad apareció asociada en el 55,5 % de los fallecidos y el 65 % tuvo lactancia mixta desde el momento de nacimiento.

Increased Intestinal Inflammation and Digestive Dysfunction in Preterm Pigs with Severe Necrotizing Enterocolitis

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Støy, Ann Cathrine Findal; Heegaard, Peter M. H.; Skovgaard, Kerstin

2017-01-01

The risk factors for necrotizing enterocolitis (NEC) are well known, but the factors involved in the different NEC presentations remain unclear. We hypothesized that digestive dysfunction and intestinal inflammation are mainly affected by severe NEC lesions. In 48 preterm pigs, the association...... between the macroscopic NEC score (range 1-6) and the expression of 48 genes related to inflammation, morphological, and digestive parameters in the distal small intestine was investigated. Only severe NEC cases (score of 5-6) were associated with the upregulation of genes involved in inflammation (CCL2...... and decreased hydrolase activity. A severe inflammatory response and digestive dysfunction are associated mainly with severe NEC. Still, it remains difficult to separate the initial causes of NEC and the later intestinal consequences of NEC in both infants and experimental models....

Antibiotic treatment preventing necrotising enterocolitis alters urinary and plasma metabolomes in preterm pigs

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Jiang, Pingping; Trimigno, Alessia; Stanstrup, Jan

2017-01-01

Necrotising enterocolitis (NEC) is a serious gut inflammatory condition in premature neonates, onset and development of which depends on the gut microbiome. Attenuation of the gut microbiome by antibiotics can reduce NEC incidence and severity. However, how the antibiotics-suppressed gut microbiome......, as shown by specific metabolites. Metabolites associated with the gut microbiome, including 3-phenyllactic acid, 4-hydroxyphenylacetic acid and phenylacetylglycine, all from phenylalanine, and three bile acids showed lower levels in the antibiotics-treated pigs where the gut microbiome was extensively...... affects the whole-body metabolism in NEC-sensitive premature neonates is unknown. In formula-fed preterm pigs, used as a model for preterm infants, plasma and urinary metabolomes were investigated by LC-MS and (1)H-NMR, with and without antibiotic treatment immediately after birth. While reducing the gut...

Elective cesarean delivery affects gut maturation and delays microbial colonization but does not increase necrotizing enterocolitis in preterm pigs

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Siggers, R. H.; Thymann, Thomas; Jensen, Bent B.

2008-01-01

Although preterm birth and formula feeding increase the risk of necrotizing enterocolitis (NEC), the influences of cesarean section (CS) and vaginal delivery (VD) are unknown. Therefore, gut characteristics and NEC incidence and severity were evaluated in preterm pigs (92% gestation) delivered...... by CS or VD. An initial study showed that newborn CS pigs (n 6) had decreased gastric acid secretion, absorption of intact proteins, activity of brush-border enzymes and pancreatic hydrolases, plasma cortisol, rectal temperature, and changes in blood chemistry, indicating impaired respiratory function...

Aspectos radiológicos relevantes no diagnóstico da enterocolite necrosante e suas complicações Relevant radiological findings for the diagnosis of necrotizing enterocolitis and its complications

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Beatriz Regina Alvares

2007-04-01

Full Text Available A enterocolite necrosante representa uma das emergências gastrintestinais mais freqüentes e graves no período neonatal. Na suspeita clínica dessa doença, o exame radiológico simples de abdome é um procedimento de rotina, desempenhando um papel fundamental no diagnóstico, acompanhamento e detecção de complicações. No presente trabalho, realizamos uma revisão da literatura pertinente e descrevemos os achados radiológicos da enterocolite necrosante, ilustrados com casos do nosso serviço. Concluímos que o diagnóstico radiológico da enterocolite necrosante realizado em todas as suas etapas, contribui para uma conduta terapêutica imediata, reduzindo as complicações e aumentando a sobrevida dos pacientes.Necrotizing enterocolitis is one of the most frequent and severe gastrointestinal emergencies occurring in the neonatal period. Once necrotizing enterocolitis is suspected a simple abdominal x-ray is a routine examination and this film will play an essential role in the diagnosis of the disease and the follow-up care of the patient, as well as in the detection of complications. In the present study we reviewed the pertinent literature and described the radiological findings, illustrated with cases from our institution. We concluded that the radiological diagnosis of necrotizing enterocolitis done at all stages contributes for an immediate therapeutic management, reducing the complications and improving the patient's survival.

Nutritional modulation of the gut microbiota and immune system in preterm neonates susceptible to necrotizing enterocolitis

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Siggers, Richard H.; Siggers, Jayda; Thymann, Thomas

2011-01-01

on the nutritional, microbial and immunological interactions during the early feeding-induced mucosal dysfunction and later NEC development. We show that introduction of suboptimal enteral formula diets, coupled with parenteral nutrition, predispose to disease, while advancing amounts of mother's milk from birth...... (particularly colostrum) protects against disease. Hence, the transition from parenteral to enteral nutrition shortly after birth plays a pivotal role to secure gut growth, digestive maturation and an appropriate response to bacterial colonization in the sensitive gut of preterm neonates.......The gastrointestinal inflammatory disorder, necrotizing enterocolitis (NEC), is among the most serious diseases for preterm neonates. Nutritional, microbiological and immunological dysfunctions all play a role in disease progression but the relationship among these determinants is not understood...

Analysis of the association between necrotizing enterocolitis and transfusion of red blood cell in very low birth weight preterm infants

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Seon-Yeong Bak

2013-03-01

Full Text Available Purpose: To investigate the association between necrotizing enterocolitis (NEC and red blood cell transfusions in very low birth weight (VLBW preterm infants. Methods: We studied were 180 VLBW preterm infants who were admitted to the neonatal intensive care unit of CHA Gangnam Hospital from January of 2006 to December of 2009. The subjects were divided into 2 groups: an NEC group (greater than stage II on the modified Bell’s criteria and a control group (less than stage II on the modified Bell’s critieria. We defined red blood cell transfusion before NEC diagnosis as the frequency of transfusion until NEC diagnosis (mean day at NEC diagnosis, day 18 in the NEC group and the frequency of transfusion until 18 days after birth in the control group. Results: Of the 180 subjects, 18 (10% belonged to the NEC group, and 14 (78% of these 18 patients had a history of transfusion before NEC diagnosis. The NEC group received 3.1±2.9 transfusions, and the control group received 1.0±1.1 transfusions before the NEC diagnosis (P =0.005. In a multivariate logistic regression corrected for gestational age, Apgar score at 1 minute, the presence of respiratory distress syndrome, patent ductus arteriosus, premature rupture of membrane, disseminated intravascular coagulopathy and death were confounding factors. The risk of NEC increased 1.63 times (95% confidence interval, 1.145 to 2.305; P =0.007 with transfusion before the NEC diagnosis. Conclusion: The risk for NEC increased significantly with increased transfusion frequency before the NEC diagnosis.

Necrotizing Enterocolitis: Old Problem with New Hope

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Shu-Fen Wu

2012-06-01

Full Text Available The incidence of necrotizing enterocolitis (NEC and mortality rate associated with this disease are not decreasing despite more than three decades of intensive research investigation and advances in neonatal intensive care. Although the etiology of NEC is not clearly elucidated, the most accepted hypothesis at present is that enteral feeding in the presence of intestinal hypoxia-ischemia-reperfusion, and colonization with pathogens provokes an inappropriately accentuated inflammatory response by the immature intestinal epithelial cells of the preterm neonate. However, delayed colonization of commensal flora with dysbiotic flora with a predominance of pathologic microorganisms plays a fundamental role in the pathogenesis of NEC. Recent studies have further identified that NEC infants have less diverse flora compared to age-matched controls without NEC. Increased gastric residual volume may be an early sign of NEC. An absolute neutrophil count of <1.5 × 109/L and platelets below 100 × 109/L are associated with an increased risk for mortality and gastrointestinal morbidity. Nonspecific supportive medical management should be initiated promptly. Sudden changes in vital signs such as tachycardia or impending shock may indicate perforation. A recent meta-analysis investigating using probiotics for prevention of NEC with a total of 2176 preterm very low birth weight infants found a success rate of just 1/25. Careful monitoring of the residual volume, and of serious changes in hemograms and vital signs may help in early diagnosis and prediction of when to perform medical or early surgical intervention. In term of prevention, administration of oral probiotics containing Bifidobacterium and Lactobacillus is a simple and safe method that attempts to early establish of commensal flora balance to inhibit pathogenic flora and an inflammatory response.

Incidence of neonatal necrotising enterocolitis in high-income countries: a systematic review.

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Battersby, Cheryl; Santhalingam, Tharsika; Costeloe, Kate; Modi, Neena

2018-03-01

To conduct a systematic review of neonatal necrotising enterocolitis (NEC) rates in high-income countries published in peer-reviewed journals. We searched MEDLINE, Embase and PubMed databases for observational studies published in peer-reviewed journals. We selected studies reporting national, regional or multicentre rates of NEC in 34 Organisation for Economic Co-operation and Development countries. Two investigators independently screened studies against predetermined criteria. For included studies, we extracted country, year of publication in peer-reviewed journal, study time period, study population inclusion and exclusion criteria, case definition, gestation or birth weight-specific NEC and mortality rates. Of the 1888 references identified, 120 full manuscripts were reviewed, 33 studies met inclusion criteria, 14 studies with the most recent data from 12 countries were included in the final analysis. We identified an almost fourfold difference, from 2% to 7%, in the rate of NEC among babies born REVIEWS REGISTRATION NUMBER: CRD42015030046. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Efficacy of Saccharomyces boulardii on necrotizing enterocolitis or sepsis in very low birth weight infants: a randomised controlled trial.

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Serce, Ozge; Benzer, Derya; Gursoy, Tugba; Karatekin, Guner; Ovali, Fahri

2013-12-01

Probiotics have strain specific effects and the effects of fungi in preventing diseases in preterm infants have been investigated poorly. Saccharomyces boulardii is a yeast which acts both as a probiotic and a polyamine producer. The objective of this study was to investigate the efficacy of S. boulardii in preventing necrotizing enterocolitis (NEC) or sepsis in very low birth weight infants. A prospective, double blind, placebo controlled trial was conducted in preterm infants (≤ 32 GWs, ≤ 1500 g birth weight). They were randomized either to receive feeding supplementation with S. boulardii 50 mg/kg every 12 h or placebo, starting with the first feed until discharged. Necrotizing enterocolitis (NEC) or sepsis and NEC or death. Birth weight and gestational age of the study (n = 104) and the control (n = 104) groups were 1126 ± 232 vs 1162 ± 216 g and 28.8 ± 2.2 vs 28.7 ± 2.1 weeks, respectively. Neither the incidence of stage ≥ 2 NEC or death nor stage ≥ 2 NEC or late onset culture proven sepsis was significantly lower in the study group when compared with the control group (9.6% vs 7.7%, p = 0.62; 28.8% vs 23%, p = 0.34). Time to reach 100 mL/kg/day of enteral feeding (11.9 ± 7 vs 12.6 ± 7 days, p = 0.37) was not different between the groups. Saccharomyces boulardii did not decrease the incidence of NEC or sepsis. © 2013 Elsevier Ireland Ltd. All rights reserved.

Latent Lymphocytic Enterocolitis Associated with Celiac Disease Manifesting after Resection for Colon Cancer: Case Report and Review of the Literature

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Vikram Tangri

2008-01-01

Full Text Available Lymphocytic colitis, a cause of chronic watery diarrhea, is histologically characterized by increased intraepithelial lymphocytosis. Studies have associated this disorder with celiac disease, although there are no reports of patients with both lymphocytic colitis and colon cancer. The present case report describes a patient with lymphocytic colitis, which manifested five years after he presented with a cecal adenocarcinoma, and three years following a diagnosis of celiac disease. Pathological review of his initial resection specimen demonstrated lymphocytic enterocolitis, indicating a five-year latency in the presentation of this disease.

The Microbiome and Metabolome of Preterm Infant Stool Are Personalized and Not Driven by Health Outcomes, Including Necrotizing Enterocolitis and Late-Onset Sepsis.

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Wandro, Stephen; Osborne, Stephanie; Enriquez, Claudia; Bixby, Christine; Arrieta, Antonio; Whiteson, Katrine

2018-06-27

The assembly and development of the gut microbiome in infants have important consequences for immediate and long-term health. Preterm infants represent an abnormal case for bacterial colonization because of early exposure to bacteria and frequent use of antibiotics. To better understand the assembly of the gut microbiota in preterm infants, fecal samples were collected from 32 very low birth weight preterm infants over the first 6 weeks of life. Infant health outcomes included health, late-onset sepsis, and necrotizing enterocolitis (NEC). We characterized bacterial compositions by 16S rRNA gene sequencing and metabolomes by untargeted gas chromatography-mass spectrometry. Preterm infant fecal samples lacked beneficial Bifidobacterium spp. and were dominated by Enterobacteriaceae , Enterococcus , and Staphylococcus organisms due to nearly uniform antibiotic administration. Most of the variance between the microbial community compositions could be attributed to the baby from which the sample derived (permutational multivariate analysis of variance [PERMANOVA] R 2 = 0.48, P PERMANOVA R 2 = 0.43, P < 0.001) and weakly associated with bacterial composition (Mantel statistic r = 0.23 ± 0.05, P < 0.05). No measured metabolites were found to be associated with necrotizing enterocolitis, late-onset sepsis, or a healthy outcome. Overall, preterm infant gut microbial communities were personalized and reflected antibiotic usage. IMPORTANCE Preterm infants face health problems likely related to microbial exposures, including sepsis and necrotizing enterocolitis. However, the role of the gut microbiome in preterm infant health is poorly understood. Microbial colonization differs from that of healthy term babies because it occurs in the NICU and is often perturbed by antibiotics. We measured bacterial compositions and metabolomic profiles of 77 fecal samples from 32 preterm infants to investigate the differences between microbiomes in health and disease. Rather than finding

Preterm infants with necrotising enterocolitis demonstrate an unbalanced gut microbiota.

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Itani, Tarek; Ayoub Moubareck, Carole; Melki, Imad; Rousseau, Clotilde; Mangin, Irène; Butel, Marie-José; Karam-Sarkis, Dolla

2018-01-01

This Lebanese study tested the hypothesis that differences would exist in the gut microbiota of preterm infants with and without necrotising enterocolitis (NEC), as reported in Western countries. This study compared 11 infants with NEC and 11 controls, all born at 27-35 weeks, in three neonatal intensive care units between January 2013 and March 2015. Faecal samples were collected at key time points, and microbiota was analysed by culture, quantitative PCR (qPCR) and temperature temporal gel electrophoresis (TTGE). The cultures revealed that all preterm infants were poorly colonised and harboured no more than seven species. Prior to NEC diagnosis, significant differences were observed by qPCR with a higher colonisation by staphylococci (p = 0.034) and lower colonisations by enterococci (p = 0.039) and lactobacilli (p = 0.048) in the NEC group compared to the healthy controls. Throughout the study, virtually all of the infants were colonised by Enterobacteriaceae at high levels. TTGE analysis revealed no particular clusterisation, showing high interindividual variability. The NEC infants were poorly colonised with no more than seven species, and the controls had a more diversified and balanced gut microbiota. Understanding NEC aetiology better could lead to more effective prophylactic interventions and a reduced incidence. ©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

Therapeutic Use of Prebiotics, Probiotics, and Postbiotics to Prevent Necrotizing Enterocolitis: What is the Current Evidence?

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Patel, Ravi Mangal; Denning, Patricia Wei

2013-01-01

Synopsis Necrotizing enterocolitis (NEC) is a leading cause of neonatal morbidity and mortality and preventative therapies that are both effective and safe are urgently needed. Current evidence from therapeutic trials suggests that probiotics are effective in decreasing NEC in preterm infants and probiotics are currently the most promising therapy on the horizon for this devastating disease. However, concerns regarding safety and optimal dosing have limited the widespread adoption of routine clinical use of probiotics in preterm infants. In addition, prebiotics and postbiotics may be potential alternatives or adjunctive therapies to the administration of live microorganisms, although studies demonstrating their clinical efficacy in preventing NEC are lacking. This review summarizes the current evidence regarding the use of probiotics, prebiotics and postbiotics in the preterm infant, including its therapeutic role in preventing NEC. PMID:23415261

Diet-Dependent Effects of Minimal Enteral Nutrition on Intestinal Function and Necrotizing Enterocolitis in Preterm Pigs

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Cilieborg, Malene Skovsted; Boye, Mette; Thymann, Thomas

2011-01-01

Background: A rapid advance in enteral feeding is associated with necrotizing enterocolitis (NEC) in preterm infants. Therefore, minimal enteral nutrition (MEN) combined with parenteral nutrition (PN) is common clinical practice, but the effects on NEC and intestinal function remain poorly...... characterized. It was hypothesized that a commonly used MEN feeding volume (16-24 mL/kg/d) prevents NEC and improves intestinal structure, function, and microbiology in preterm pigs. Methods: After preterm birth pigs were stratified into 4 nutrition intervention groups that received the following treatments: (1...... later formula-induced gut dysfunction and NEC. However, in CF pigs, intestinal lesions were restricted to the colon, compared with all regions in OF and FF pigs, which indicated proximal protection of colostrum MEN. Bacterial composition was not affected by MEN, diet, or NEC outcomes, but bacterial load...

Lactoferrin and neonatology - role in neonatal sepsis and necrotizing enterocolitis: present, past and future.

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Sharma, Deepak; Shastri, Sweta

2016-03-01

Neonatal sepsis and necrotizing enterocolitis (NEC) are two most important neonatal problems in nursery which constitute the bulk of neonatal mortality and morbidity. Inflammatory mediators secondary to sepsis and NEC increases morbidity, by affecting various system of body like lung, brain and eye, thus causing long term implications. Lactoferrin (LF) is a component of breast milk and multiple actions that includes antimicrobial, antiviral, anti-fungal and anti-cancer and various other actions. Few studies have been completed and a number of them are in progress for evaluation of efficacy and safety of LF in the prevention of neonatal sepsis and NEC in field of neonatology. In future, LF prophylaxis and therapy may have a significant impact in improving clinical outcomes of vulnerable preterm neonates. This review analyse the role of lactoferrin in prevention of neonatal sepsis and NEC, with emphasis on mechanism of action, recent studies and current studies going on around the globe.

The role of nitric oxide in an experimental necrotising enterocolitis model

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Muazez Cevik

2013-01-01

Full Text Available Background: Necrotising enterocolitis (NEC causes a significant life-threatening gastrointestinal system (GIS disease with severe mortality and morbidity, particularly in premature infants. Nitric oxide (NO has many functions in the GIS. Therefore, in the present study, we evaluated the effects of NO in experimentally induced NEC of newborn 1-day-old rats following hypoxia/reoxygenation (HR. Materials and Methods: Thirty Wistar albino rats (weight, 5-8 g were randomly divided into three groups: group 1 (HR, group 2 (HR + nitroglycerine, and group 3 (control. HR was achieved by placing the rat in carbon dioxide (CO2 for five minutes at 22°C, which was followed by five minutes of 100% oxygen. After HR, nitroglycerine was administered for three days at 50 μg/Kg/day. On day 4, the rats were decapitated and the intestines between the duodenum and sigmoid colon were resected and histopathologically examined. Results: The histopathological findings of groups 1 and 2 were characteristic of NEC. Intestinal injury in group 1 was significantly more prevalent than that in group 2 (χ2 = 21.55, P = 0.000. The intestinal injury score in group 3 was significantly lower than that in the other groups (P < 0.05. Conclusions: NO treatment was effective for treating experimentally induced NEC.

Prematurity reduces functional adaptation to intestinal resection in piglets

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Aunsholt, Lise; Thymann, Thomas; Qvist, Niels

2015-01-01

Background: Necrotizing enterocolitis and congenital gastrointestinal malformations in infants often require intestinal resection, with a subsequent risk of short bowel syndrome (SBS). We hypothesized that immediate intestinal adaptation following resection of the distal intestine with placement ...

Enteric serotonin and oxytocin: endogenous regulation of severity in a murine model of necrotizing enterocolitis.

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Gross Margolis, Kara; Vittorio, Jennifer; Talavera, Maria; Gluck, Karen; Li, Zhishan; Iuga, Alina; Stevanovic, Korey; Saurman, Virginia; Israelyan, Narek; Welch, Martha G; Gershon, Michael D

2017-11-01

Necrotizing enterocolitis (NEC), a gastrointestinal inflammatory disease of unknown etiology that may also affect the liver, causes a great deal of morbidity and mortality in premature infants. We tested the hypothesis that signaling molecules, which are endogenous to the bowel, regulate the severity of intestinal and hepatic damage in an established murine NEC model. Specifically, we postulated that mucosal serotonin (5-HT), which is proinflammatory, would exacerbate experimental NEC and that oxytocin (OT), which is present in enteric neurons and is anti-inflammatory, would oppose it. Genetic deletion of the 5-HT transporter (SERT), which increases and prolongs effects of 5-HT, was found to increase the severity of systemic manifestations, intestinal inflammation, and associated hepatotoxicity of experimental NEC. In contrast, genetic deletion of tryptophan hydroxylase 1 (TPH1), which is responsible for 5-HT biosynthesis in enterochromaffin (EC) cells of the intestinal mucosa, and TPH inhibition with LP-920540 both decrease the severity of experimental NEC in the small intestine and liver. These observations suggest that 5-HT from EC cells helps to drive the inflammatory damage to the gut and liver that occurs in the murine NEC model. Administration of OT decreased, while the OT receptor antagonist atosiban exacerbated, the intestinal inflammation of experimental NEC. Data from the current investigation are consistent with the tested hypotheses-that the enteric signaling molecules, 5-HT (positively) and OT (negatively) regulate severity of inflammation in a mouse model of NEC. Moreover, we suggest that mucosally restricted inhibition of 5-HT biosynthesis and/or administration of OT may be useful in the treatment of NEC. NEW & NOTEWORTHY Serotonin (5-HT) and oxytocin reciprocally regulate the severity of intestinal inflammation and hepatotoxicity in a murine model of necrotizing enterocolitis (NEC). Selective depletion of mucosal 5-HT through genetic deletion or

Severe forms of food allergy.

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Sarinho, Emanuel; Lins, Maria das Graças Moura

To guide the diagnostic and therapeutic management of severe forms of food allergy. Search in the Medline database using the terms "severe food allergy," "anaphylaxis and food allergy," "generalized urticaria and food allergy," and "food protein-induced enterocolitis syndrome" in the last ten years, searching in the title, abstract, or keyword fields. Food allergy can be serious and life-threatening. Milk, eggs, peanuts, nuts, walnuts, wheat, sesame seeds, shrimp, fish, and fruit can precipitate allergic emergencies. The severity of reactions will depend on associated cofactors such as age, drug use at the onset of the reaction, history and persistence of asthma and/or severe allergic rhinitis, history of previous anaphylaxis, exercise, and associated diseases. For generalized urticaria and anaphylaxis, intramuscular epinephrine is the first and fundamental treatment line. For the treatment in acute phase of food-induced enterocolitis syndrome in the emergency setting, prompt hydroelectrolytic replacement, administration of methylprednisolone and ondansetron IV are necessary. It is important to recommend to the patient with food allergy to maintain the exclusion diet, seek specialized follow-up and, in those who have anaphylaxis, to emphasize the need to carry epinephrine. Severe food allergy may occur in the form of anaphylaxis and food-protein-induced enterocolitis syndrome, which are increasingly observed in the pediatric emergency room; hence, pediatricians must be alert so they can provide the immediate diagnosis and treatment. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Long-term outcome in patients with short bowel syndrome after longitudinal intestinal lengthening and tailoring.

Science.gov (United States)

Reinshagen, K; Kabs, C; Wirth, H; Hable, N; Brade, J; Zahn, K; Hagl, C; Jester, I; Waag, K L

2008-11-01

Longitudinal intestinal lengthening and tailoring (LILT) is a well-established surgical treatment for short bowel syndrome. It has been shown to enhance peristalsis, decrease bacterial overgrowth, and extend mucosal contact time for nutrients. We present the results of a long-term follow-up of patients who underwent LILT and define prognostic parameters for the survival of these patients. Between 1987 and 2006, 53 patients underwent LILT in our institution. The main diagnoses were gastroschisis, intestinal volvulus, intestinal atresias, and necrotizing enterocolitis. LILT was performed at a mean age of 24 months (range 4144 months). The follow-up time was 79.76 months (range 6234 months). After LILT, 41 of 53 patients survived, and 36 of 41 surviving patients were successfully weaned from parenteral nutrition (PN). In long-term follow-up 79% stayed free of PN. The overall survival rate was 77.36%. Weight gain occurred in 58% of the patients after LILT. The quality of life after LILT is on a high level, with most patients having normal physical strength and participating in normal social life and education. Prognostic factors for survival after LILT in short bowel syndrome are length of small intestine (0.06582 + 0.0131 x bowel cm), length of large bowel (P = 0.039), preoperative liver function, and successful weaning from PN within 18 months postoperatively (P = 0.0032). Patients undergoing LILT in short bowel syndrome have a high survival rate, weight gain, and a high quality of life. Autologous gastrointestinal reconstruction remains therefore the first choice in the treatment of patients with short bowel syndrome.

Enteral but not parenteral antibiotics enhance gut function and prevent necrotizing enterocollitis in forumula-fed newborn preterm pigs

DEFF Research Database (Denmark)

Birck, Malene M; Nguyen, Duc Ninh; Cilieborg, Malene Skovsted

2016-01-01

, relative to CON pigs (P pigs were intermediate with few affected parameters (reduced lactic acid levels and density and adherence of Gram-positive bacteria, relative to CON pigs, P antimicrobial resistance following the treatments. We......Preterm infants are susceptible to infection and necrotizing enterocolitis (NEC) and are often treated with antibiotics. Simultaneous administration of enteral and parenteral antibiotics during the first days after preterm birth prevents formula-induced NEC lesions in pigs, but it is unknown which...... administration route is most effective. We hypothesized that only enteral antibiotics suppress gut bacterial colonization and NEC progression in formula-fed preterm pigs. Caesarean-delivered preterm pigs (90–92% of gestation) were fed increasing amounts of infant formula from birth to day 5 and given saline (CON...

Severe forms of food allergy

Directory of Open Access Journals (Sweden)

Emanuel Sarinho

2017-11-01

Conclusion: Severe food allergy may occur in the form of anaphylaxis and food‐protein‐induced enterocolitis syndrome, which are increasingly observed in the pediatric emergency room; hence, pediatricians must be alert so they can provide the immediate diagnosis and treatment.

Prospective evaluation of radionuclide scanning in detection of intestinal necrosis in neonatal necrotizing enterocolitis

International Nuclear Information System (INIS)

Haase, G.M.; Sfakianakis, G.N.; Lobe, T.E.; Boles, E.T.

1981-01-01

The ability of external imaging to demonstrate intestinal infarction in neonatal necrotizing enterocolitis (NEC) was prospectively evaluated. The radiopharmaceutical technetium--99m diphosphonate was injected intravenously and the patients subsequently underwent abdominal scanning. Clinical patient care and interpretation of the images were entirely independent throughout the study. Of 33 studies, 7 were positive, 4 were suspicious, and 22 were negative. One false positive study detected ischemia without transmural infarction. The second false positive scan occurred postoperatively and was due to misinterpretation of the hyperactivity along the surgical incision. None of the suspicious cases had damaged bowel. The two false negative studies clearly failed to demonstrate frank intestinal necrosis. The presence of very small areas of infarction, errors in technical settings, subjective interpretation of scans and delayed clearance of the radionuclide in a critically ill neonate may all limit the accuracy of external abdominal scanning. However, in spite of an error rate of 12%, it is likely that this technique will enhance the present clinical, laboratory, and radiologic parameters of patient management in NEC

Probiotics, Prebiotics, and Synbiotics for the Prevention of Necrotizing Enterocolitis.

Science.gov (United States)

Johnson-Henry, Kathene C; Abrahamsson, Thomas R; Wu, Richard You; Sherman, Philip M

2016-09-01

Necrotizing enterocolitis (NEC) is a devastating intestinal disease in preterm infants characterized by barrier disruption, intestinal microbial dysbiosis, and persistent inflammation of the colon, which results in high mortality rates. Current strategies used to manage this disease are not sufficient, although the use of human breast milk reduces the risk of NEC. Mother's milk is regarded as a fundamental nutritional source for neonates, but pasteurization of donor breast milk affects the composition of bioactive compounds. Current research is evaluating the benefits and potential pitfalls of adding probiotics and prebiotics to pasteurized milk so as to improve the functionality of the milk and thereby reduce the burden of illness caused by NEC. Probiotics (live micro-organisms that confer health to the host) and prebiotics (nondigestible oligosaccharides that stimulate the growth of healthy bacteria) are functional foods known to mediate immune responses and modulate microbial populations in the gut. Clinical research shows strain- and compound-specific responses when probiotics or prebiotics are administered in conjunction with donor breast milk for the prevention of NEC. Despite ongoing controversy surrounding optimal treatment strategies, randomized controlled studies are now investigating the use of synbiotics to reduce the incidence and severity of NEC. Synbiotics, a combination of probiotics and prebiotics, have been proposed to enhance beneficial health effects in the intestinal tract more than either agent administered alone. This review considers the implications of using probiotic-, prebiotic-, and synbiotic-supplemented breast milk as a strategy to prevent NEC and issues that could be encountered with the preparations. © 2016 American Society for Nutrition.

Hemodynamic and permeability characteristics of acute experimental necrotizing enterocolitis

International Nuclear Information System (INIS)

Miller, M.J.; Adams, J.; Gu, X.A.; Zhang, X.J.; Clark, D.A.

1990-01-01

We examined the local hemodynamic response of intestinal loops during acute necrotizing enterocolitis (NEC) in anesthetized rabbits. NEC was induced in ileal loops by transmural injection of a solution containing casein (10 mg/ml) and calcium gluconate (50 mg/ml) acidified to pH 4.0 with propionic or acetic acid. Control loops received casein only (pH 5.0). Mucosal damage was quantified by the blood-to-lumen movement of [51Cr]EDTA, fluid shifts into the lumen, and histology. Mean arterial pressure and loop blood flow were steady over the 3-hr period, loop fluid volume decreased, and there was no evidence of necrosis or epithelial damage. In loops receiving acidified casein and calcium gluconate, there was an immediate dramatic increase in loop blood flow that returned to baseline by 50 min. In addition, loop fluid volume was dramatically increased, necrosis was noted in the form of blunting and loss of villi, and sevenfold increase in [51Cr]EDTA permeability was evident. Administration of CV 1808 (30 mg/kg/hr), a selective adenosine2 agonist, which maintained and elevated loop blood flow throughout the 3 hr protocol, failed to alter the changes in loop fluid volume or prevent necrosis. Histamine levels in loop fluid levels were significantly elevated 20-30 min after NEC induction when compared to saline controls, indicating an early activation of mucosal defenses with this luminal insult. Thus, this model of NEC is characterized by a transient, acute hyperemia, increased intestinal permeability, and histamine release. As mucosal damage was independent of ischemia and could not be prevented by vasodilatory therapy, this model supports the clinical findings that NEC is correlated with luminal factors related to feeding and independent of cardiovascular stress

High Prevalence of Neutrophil Cytoplasmic Autoantibodies in Infants with Food Protein-Induced Proctitis/Proctocolitis: Autoimmunity Involvement?

Czech Academy of Sciences Publication Activity Database

Sekerková, A.; Fuchs, M.; Cecrdlová, E.; Šváchová, V.; Kralová Lesná, I.; Stříž, I.; Tlaskalová-Hogenová, Helena

2015-01-01

Roč. 2015, September 2015 (2015), s. 902863 ISSN 2314-8861 R&D Projects: GA MZd(CZ) NT13483 Institutional support: RVO:61388971 Keywords : INDUCED ENTEROCOLITIS SYNDROME * INFLAMMATORY-BOWEL-DISEASE * BREAST-FED INFANTS Subject RIV: EE - Microbiology, Virology Impact factor: 2.812, year: 2015

Poly(ADP-Ribose) Polymerase-1: A Novel Therapeutic Target in Necrotizing Enterocolitis

Science.gov (United States)

Giannone, Peter J.; Alcamo, Alicia A.; Schanbacher, Brandon L.; Nankervis, Craig A.; Besner, Gail E.; Bauer, John A.

2011-01-01

Necrotizing enterocolitis (NEC) is the most common gastrointestinal disease of infancy, afflicting 11% of infants born 22–28 weeks gestational age. Both inflammation and oxidation may be involved in NEC pathogenesis through reactive nitrogen species production, protein oxidation and DNA damage. Poly(ADP-ribose) polymerase-1 (PARP-1) is a critical enzyme activated to facilitate DNA repair using nicotinamide adenine dinucleotide (NAD+) as a substrate. However, in the presence of severe oxidative stress and DNA damage, PARP-1 over-activation may ensue, depleting cells of NAD+ and ATP, killing them by metabolic catastrophe. Here we tested the hypothesis that NO dysregulation in intestinal epithelial cells during NEC leads to marked PARP-1 expression and that administration of a PARP-1 inhibitor (nicotinamide) attenuates intestinal injury in a newborn rat model of NEC. In this model, 56% of control pups developed NEC (any stage), versus 14% of pups receiving nicotinamide. Forty-four percent of control pups developed high-grade NEC (grades 3–4), whereas only 7% of pups receiving nicotinamide developed high-grade NEC. Nicotinamide treatment protects pups against intestinal injury incurred in the newborn rat NEC model. We speculate that PARP-1 over-activation in NEC may drive mucosal cell death in this disease and that PARP-1 may be a novel therapeutic target in NEC. PMID:21399558

Bifidobacterium breve prevents necrotising enterocolitis by suppressing inflammatory responses in a preterm rat model.

Science.gov (United States)

Satoh, T; Izumi, H; Iwabuchi, N; Odamaki, T; Namba, K; Abe, F; Xiao, J Z

2016-02-01

Necrotising enterocolitis (NEC) is associated with inflammatory responses and barrier dysfunction in the gut. In this study, we investigated the effect of Bifidobacterium breve M-16V on factors related to NEC development using an experimental rat model. Caesarean-sectioned rats were given formula milk with or without B. breve M-16V by oral gavage thrice daily, and experimental NEC was induced by exposing the rats to hypoxic conditions. Naturally delivered rats that were reared by their mother were used as healthy controls. The pathological score of NEC and the expression of molecules related to inflammatory responses and the barrier function were assessed in the ileum. B. breve M-16V reduced the pathological scores of NEC and resulted in some improvement in survivability. B. breve M-16V suppressed the increased expression of molecules related to inflammation and barrier function that resulted from NEC induction. B. breve M-16V normalised Toll-like receptor (TRL)4 expression and enhanced TLR2 expression. Our data suggest that B. breve M-16V prevents NEC development by modulating TLR expressions and suppressing inflammatory responses in a rat model.

Autistic Enterocolitis: Fact or Fiction?

Directory of Open Access Journals (Sweden)

Polymnia Galiatsatos

2009-01-01

Full Text Available Autism spectrum disorder refers to syndromes of varying severity, typified by impaired social interactions, communicative delays and restricted, repetitive behaviours and interests. The prevalence of autism spectrum disorders has been on the rise, while the etiology remains unclear and most likely multifactorial. There have been several reports of a link between autism and chronic gastrointestinal symptoms. Endoscopy trials have demonstrated a higher prevalence of nonspecific colitis, lymphoid hyperplasia and focally enhanced gastritis compared with controls. Postulated mechanisms include aberrant immune responses to some dietary proteins, abnormal intestinal permeability and unfavourable gut microflora. Two autism spectrum disorder patients with chronic intestinal symptoms and abnormal endoscopic findings are described, followed by a review of this controversial topic.

Efficacy of Probiotics Versus Placebo in the Prevention of Necrotizing Enterocolitis in Preterm Very Low Birth Weight Infants: A Double-blind Randomized Controlled Trial

International Nuclear Information System (INIS)

Chowdhury, T.; Ali, M.M.; Hossain, M. M.

2016-01-01

Objective: To evaluate the efficacy of orally administered probiotics in preventing necrotizing enterocolitis (NEC) in preterm very low birth weight (VLBW) infants. Study Design: Arandomized double blind controlled trial. Place and Duration of Study: The Paediatrics Department of Sylhet M.A.G. Osmani Medical College Hospital, Sylhet Bangladesh, from July 2012 to June 2015. Methodology: Preterm (28 - 33 weeks gestation) VLBW (birth weight 1000 - 1499 g) neonates were enrolled. The study group was fed with probiotics once daily with breast milk from first feeding, and the control group with only breast milk without the addition of probiotics. Both the groups received other standard care. The primary outcome was the development of NEC (stage II and III), categorized by modified Bell's classification. Result: In 108 neonates, development of NEC was significantly lower in the study group than that of control group [1 (1.9 percent) vs. 6 (11.5 percent); p=0.044]. Age of achievement of full oral feeding was significantly earlier in the study group than that in the control group (14.88 ±3.15 and 18.80 ±4.32 days; p < 0.001). Duration of hospital stay was significantly short in the study group compared to the control group (15.82 ±2.94 days vs. 19.57 ±4.26 days; p < 0.001). Conclusion: Probiotic supplementation reduces the frequency of necrotising enterocolitis in preterm neonates with very low birth weight. It is also associated with faster achievement of full oral feeding and short duration of hospital stay. (author)

Mode of Action of Shigella Toxin: Effects on Ribosome Structure and Function

Science.gov (United States)

1988-05-01

1974. Sindrome hemolitico uremico: reporte de 60 casos asociados a una epidemia de enterocolitis hemorragica. Revista Colombiana de Ped. Puericult. 28...1518-1521. 34. Fong, J.S.C., J-P de Chadarevian and B.S. Kaplan. 1982. Hemolytic-uremic syndrome: current concepts and management. Ped. Clin. North Am

The Role of Immunonutrients in the Prevention of Necrotizing Enterocolitis in Preterm Very Low Birth Weight Infants

Directory of Open Access Journals (Sweden)

Ping Zhou

2015-08-01

Full Text Available Necrotizing enterocolitis (NEC is a critical intestinal emergency condition, which mainly occurs in preterm very low birth weight (PVLBW infants. Despite remarkable advances in the care of PVLBW infants, with considerable improvement of the survival rate in recent decades, the incidence of NEC and NEC-related mortality have not declined accordingly. The fast progression from nonspecific signs to extensive necrosis also makes primary prevention the first priority. Recently, increasing evidence has indicated the important role of several nutrients in primary prevention of NEC. Therefore, the aim of this review is to summarize some potential immunomodulatory nutrients in the prevention of NEC, including bovine colostrum, probiotics, prebiotics (e.g., human milk oligosaccharides, long chain polyunsaturated fatty acids, and amino acids (glutamine, cysteine and N-acetylcysteine, l-arginine and l-citrulline. Based on current research evidence, probiotics are the most documented effective method to prevent NEC, while others still require further investigation in animal studies and clinical randomized controlled trials.

Probiotics, Prebiotics, and Synbiotics for the Prevention of Necrotizing Enterocolitis12

Science.gov (United States)

Wu, Richard You

2016-01-01

Necrotizing enterocolitis (NEC) is a devastating intestinal disease in preterm infants characterized by barrier disruption, intestinal microbial dysbiosis, and persistent inflammation of the colon, which results in high mortality rates. Current strategies used to manage this disease are not sufficient, although the use of human breast milk reduces the risk of NEC. Mother’s milk is regarded as a fundamental nutritional source for neonates, but pasteurization of donor breast milk affects the composition of bioactive compounds. Current research is evaluating the benefits and potential pitfalls of adding probiotics and prebiotics to pasteurized milk so as to improve the functionality of the milk and thereby reduce the burden of illness caused by NEC. Probiotics (live micro-organisms that confer health to the host) and prebiotics (nondigestible oligosaccharides that stimulate the growth of healthy bacteria) are functional foods known to mediate immune responses and modulate microbial populations in the gut. Clinical research shows strain- and compound-specific responses when probiotics or prebiotics are administered in conjunction with donor breast milk for the prevention of NEC. Despite ongoing controversy surrounding optimal treatment strategies, randomized controlled studies are now investigating the use of synbiotics to reduce the incidence and severity of NEC. Synbiotics, a combination of probiotics and prebiotics, have been proposed to enhance beneficial health effects in the intestinal tract more than either agent administered alone. This review considers the implications of using probiotic-, prebiotic-, and synbiotic-supplemented breast milk as a strategy to prevent NEC and issues that could be encountered with the preparations. PMID:27633108

Utility of ultrasound in the early diagnosis of childhood neutropenic enterocolitis

International Nuclear Information System (INIS)

Muro, D.; Sanguesa, C.; Bermudez, M.; Verdeguer, A.

1999-01-01

To determine the utility of ultrasound in the diagnosis of neutropenic enterocolitis (NEC) in children with cancer or AIDS, and to establish the differential diagnosis with respect to more common causes of acute abdomen. We studied eight patients diagnosed as having NEC over a ten-year period, gathering the clinical data and imaging studied for each. Seven of the children were cancer patients and the remaining child had AIDS. The imaging methods used were plain radiography, B-mode ultrasound and color Doppler ultrasound: in addition, one patient underwent a barium enema. All eight patients presented abdominal pain, predominantly in right iliac fossa, and fever; the neutrophil count was under 100/mm''3. The most common plain abdominal X-ray image was the absence of air in right iliac fossa and dilation of the small bowel loops. One child presented submucosal and subserosal ectopic air colon and cecum, air in the intrahepatic portal system and in peritoneum. Abdominal ultrasound revealed thickening of the cecal mucosa in every case, and color Doppler ultrasound disclosed increased vascularization of the intestinal wall. Six children received medical treatment and two underwent surgery. Three patients died, one a few hours after the surgical procedure and the other two a few days after onset of NEC and initiation of medical treatment. Abdominal ultrasound is the best imaging technique for early diagnosis of NEC and assessment of the response to medical treatment, Moreover, it can be performed at bedside, making it unnecessary to move the patient from his room. (Author) 26 refs

Enterocolic lymphocytic phlebitis of the cecal pole and appendix vermiformis with increase of IgG4-positive plasma cells.

Science.gov (United States)

Comtesse, Sarah; Friemel, Juliane; Fankhauser, René; Weber, Achim

2014-01-01

Here we describe the clinicopathological course of a 20-year-old female patient with enterocolic lymphocytic phlebitis (ELP) of the appendix vermiformis and cecal pole with increase of IgG4-positive plasma cells. The patient presented with acute abdomen, suspicious of acute appendicitis. Diagnostic laparoscopy showed tumefaction of the cecal pole and appendix vermiformis. Histologic examination revealed mural thickening and a dense lymphoplasmocytic, partly obliterative infiltrate of the veins with sparing of the arteries, diagnostic of ELP. In addition, we found an elevated number of IgG4-positive plasma cells blended in with the lymphocytes. The IgG4-to-IgG ratio accounted for >40 %. This case meets the histopathological criteria requested for IgG4-related disease (IgG4-RD) and thus opens the possibility that ELP might be part of the IgG4-RD spectrum.

Usefulness of Ultrasound Examinations in the Diagnostics of Necrotizing Enterocolitis

International Nuclear Information System (INIS)

Staryszak, Joanna; Stopa, Joanna; Kucharska-Miąsik, Iwona; Osuchowska, Magdalena; Guz, Wiesław; Błaż, Witold

2015-01-01

Necrotizing enterocolitis (NEC) is one of the most serious disorders of gastrointestinal tract during neonatal period. Early diagnosis and adequate treatment are essential in the presence of clinical suspicion of NEC. Plain abdominal radiography is currently the modality of choice for initial evaluation of gastrointestinal tract in neonates. However, when the diagnosis is uncertain, abdominal ultrasound with bowel assessment might be an important complementary examination. The aim of the study was to evaluate usefulness of ultrasound in the diagnosis of NEC and its value for implementation of proper treatment. The data of nine neonates diagnosed with NEC, hospitalized at the Provincial Hospital No. 2 in Rzeszow in the period from September 2009 to April 2013 was retrospectively analyzed. Apart from abdominal radiography, abdominal ultrasound with bowel assessment was performed in all nine cases. Imaging findings, epidemiological data, coexisting risk factors and disease course were assessed. Most children in the group were preterm neonates. Findings in plain abdominal radiography were normal or nonspecific. A wider spectrum of findings was demonstrated in all ultrasound examinations and intestinal pneumatosis, a pathognomonic sign for NEC, was more frequently noted than in plain abdominal x-ray. Most children were treated by surgical intervention with resection of necrotic bowel loops and in more than half of the cases location of changes identified during surgery was concordant with ultrasonographic findings. Abdominal ultrasound examination might be helpful in the diagnosis of NEC, especially when plain abdominal radiography findings do not correlate with clinical symptoms. However, abdominal radiography is still considered the modality of choice. The range of morphological changes detectable on ultrasound examination is much wider than in plain abdominal radiography. Ultrasound examination allows for more accurate assessment of changes within intestines and

The timing of ostomy closure in infants with necrotizing enterocolitis: a systematic review.

Science.gov (United States)

Struijs, Marie-Chantal; Sloots, Cornelius E J; Hop, Wim C J; Tibboel, Dick; Wijnen, Rene M H

2012-07-01

The optimal timing of ostomy closure is a matter of debate. We performed a systematic review of outcomes of early ostomy closure (EC, within 8 weeks) and late ostomy closure (LC, after 8 weeks) in infants with necrotizing enterocolitis. PubMed, EMbase, Web-of-Science, and Cinahl were searched for studies that detailed time to ostomy closure, and time to full enteral nutrition (FEN) or complications after ostomy closure. Patients with Hirschsprung's disease or anorectal malformations were excluded. Analysis was performed using SPSS 17 and RevMan 5. Of 778 retrieved articles, 5 met the inclusion criteria. The median score for study quality was 9 [range 8-14 on a scale of 0 to 32 points (Downs and Black, J Epidemiol Community Health 52:377-384, 1998)]. One study described mean time to FEN: 19.1 days after EC (n = 13) versus 7.2 days after LC (n = 24; P = 0.027). Four studies reported complication rates after ostomy closure, complications occurred in 27% of the EC group versus 23% of the LC group. The combined odds ratio (LC vs. EC) was 1.1 [95% CI 0.5, 2.5]. Evidence that supports early or late closure is scarce and the published articles are of poor quality. There is no significant difference between EC versus LC in the complication rate. This systematic review supports neither early nor late ostomy closure.

The science and necessity of using animal models in the study of necrotizing enterocolitis.

Science.gov (United States)

Ares, Guillermo J; McElroy, Steven J; Hunter, Catherine J

2018-02-01

Necrotizing enterocolitis (NEC) remains one of the highest causes of mortality and of acute and long-term morbidity in premature infants. Multiple factors are involved in the pathophysiology of NEC including the immaturity of the immune system and the complex changing composition of the intestinal microbiome. This is compounded by the fact that the premature infant should ideally still be a developing fetus and has an immature intestinal tract. Because these complexities are beyond the scope of studies in single-cell cultures, animal models are absolutely essential to understand the mechanisms involved in the pathophysiology of NEC and the effects of inflammation on the immature intestinal tract. To this end, investigators have utilized many different species (e.g., rats, mice, rabbits, quails, piglets, and non-human primates) and conditions to develop models of NEC. Each animal has distinct advantages and drawbacks related to its preterm viability, body size, genetic variability, and cost. The choice of animal model is strongly influenced by the scientific question being addressed. While no model perfectly mimics human NEC, each has greatly improved our understanding of disease. Examples of recent discoveries in NEC pathogenesis and prevention underscore the importance of continued animal research in NEC. Copyright © 2018 Elsevier Inc. All rights reserved.

Severe forms of food allergy

Directory of Open Access Journals (Sweden)

Emanuel Sarinho

Full Text Available Abstract Objectives: To guide the diagnostic and therapeutic management of severe forms of food allergy. Data sources: Search in the Medline database using the terms “severe food allergy,” “anaphylaxis and food allergy,” “generalized urticaria and food allergy,” and “food protein-induced enterocolitis syndrome” in the last ten years, searching in the title, abstract, or keyword fields. Summary of data: Food allergy can be serious and life-threatening. Milk, eggs, peanuts, nuts, walnuts, wheat, sesame seeds, shrimp, fish, and fruit can precipitate allergic emergencies. The severity of reactions will depend on associated cofactors such as age, drug use at the onset of the reaction, history and persistence of asthma and/or severe allergic rhinitis, history of previous anaphylaxis, exercise, and associated diseases. For generalized urticaria and anaphylaxis, intramuscular epinephrine is the first and fundamental treatment line. For the treatment in acute phase of food-induced enterocolitis syndrome in the emergency setting, prompt hydroelectrolytic replacement, administration of methylprednisolone and ondansetron IV are necessary. It is important to recommend to the patient with food allergy to maintain the exclusion diet, seek specialized follow-up and, in those who have anaphylaxis, to emphasize the need to carry epinephrine. Conclusion: Severe food allergy may occur in the form of anaphylaxis and food-protein-induced enterocolitis syndrome, which are increasingly observed in the pediatric emergency room; hence, pediatricians must be alert so they can provide the immediate diagnosis and treatment.

The effect of bovine colostrum products on intestinal dysfunction and inflammation in a preterm pig model of necrotizing enterocolitis

DEFF Research Database (Denmark)

Støy, Ann Cathrine Findal

Necrotizing enterocolitis (NEC), primarily seen in preterm infants, is associated with high morbidity and mortality. The pathogenesis is not fully understood but risk factors include prematurity, enteral feeding (especially with milk formula), and the intestinal microbiota. Mother’s milk, rich...... in bioactive factors, has a protective effect against NEC, but not all preterm infants are able to receive mother’s milk. The overall aim of this thesis was to investigate if bovine colostrum (BC), also rich in bioactive factors, could serve as an alternative to mother’s milk. A preterm pig model of NEC...... formula. All three BC products maintained trophic and anti-inflammatory effects on the immature pig intestine. A simple and standardized system was required to investigate the effects of milk formula versus BC on intestinal epithelial cells. In Study III, the IPEC-J2 cell line was evaluated as an in vitro...

CT features of neutropenic enterocolitis in adult patients with hematological diseases undergoing chemotherapy

Energy Technology Data Exchange (ETDEWEB)

Vogel, M.N.; Brodoefel, H.; Claussen, C.D.; Horger, M. [Tuebingen Univ. (Germany). Dept. of Diagnostic and Interventional Radiology; Goeppert, B. [Heidelberg Univ. (Germany). Inst. of Pathology; Maksimovic, O.; Faul, C. [Tuebingen Univ. (Germany). Dept. of Internal Medicine-Oncology

2010-12-15

Purpose: This study investigates the features of neutropenic enterocolitis (NE) in adults. Materials and Methods: Chart and radiology report reviews were used to identify neutropenic patients with hematological diseases undergoing chemotherapy, who had CT scans for the clarification of abdominal symptoms between October 2003 and October 2009. Patients with any cause for enteritis other than NE were excluded. The scans were analyzed with respect to imaging features and location. Morphological findings were correlated with clinical data. Results: Thirty-one patients with NE (median age 46 years; range 20 - 75) could be identified. Wall thickening and hyperemia could be found in all bowel segments from jejunum to rectum. The right hemicolon was the most frequent location in 19 patients (61 %). Involvement was generalized in 6 patients (19 %) and segmental in 25 cases (81 %). The longer the duration of neutropenia, the more likely generalized involvement of the bowel was. In 8 patients who underwent CT follow-up, the appearance of bowel segments had completely (n = 5) or partially (n = 3) returned to normal at the latest 14 days after the initial diagnosis. Eight patients (26 %) died 1 - 78 days after NE, 7 of who had previously recovered from NE. Conclusion: CT findings are useful for the diagnosis of NE and should be considered even in the presence of isolated small bowel involvement. The terms NE and typhlitis should thus no longer be used synonymously. (orig.)

Changes in plasma and urinary nitrite after birth in premature infants at risk for necrotizing enterocolitis

Science.gov (United States)

Pun, Priti; Jones, Jesica; Wolfe, Craig; Deming, Douglas D.; Power, Gordon G.; Blood, Arlin B.

2016-01-01

Background Plasma nitrite serves as a reservoir of nitric oxide (NO) bioactivity. Because nitrite ingestion is markedly lower in newborns than adults, we hypothesized plasma nitrite levels would be lower in newborns than in adults, and that infants diagnosed with necrotizing enterocolitis (NEC), a disease characterized by ischemia and bacterial invasion of intestinal walls, would have lower levels of circulating nitrite in the days prior to diagnosis. Methods Single blood and urine samples were collected from 9 term infants and 12 adults, 72 preterm infants every 5 d for 3 wk, and from 13 lambs before and after cord occlusion. Results Nitrite fell 50% relative to cord levels in the first day after birth; and within 15 min after cord occlusion in lambs. Urinary nitrite was higher in infants than adults. Plasma and urinary nitrite levels in infants who developed NEC were similar to those of preterm control infants on days 1 and 5, but significantly elevated at 15 and 20 d after birth. Conclusion Plasma nitrite falls dramatically at birth while newborn urinary nitrite levels are significantly greater than adults. Acute NEC is associated with elevated plasma and urinary nitrite levels. PMID:26539663

Saccharomyces boulardii for prevention of necrotizing enterocolitis in preterm infants: a randomized, controlled study.

Science.gov (United States)

Demirel, Gamze; Erdeve, Omer; Celik, Istemi Han; Dilmen, Ugur

2013-12-01

To evaluate the efficacy of orally administered Saccharomyces boulardii (S. boulardii) for reducing the incidence and severity of necrotizing enterocolitis (NEC) in very low-birth-weight (VLBW) infants. A prospective, randomised controlled trial was conducted in infants with gestational age ≤32 weeks and birth weight ≤1500 g. The study group received S. boulardii supplementation, and the control group did not. The primary outcomes were death or NEC (Bell's stage ≥2), and secondary outcomes were feeding intolerance and clinical or culture-proven sepsis. A total of 271 infants were enrolled in the study, 135 in the study group and 136 in the control group. There was no significant difference in the incidence of death (3.7% vs. 3.6%, 95% CI of the difference, -5.20-5.25; p = 1.0) or NEC (4.4% vs. 5.1%, 95% CI, -0.65-5.12; p = 1.0) between the groups. However, feeding intolerance and clinical sepsis were significantly lower in the probiotic group compared with control. Although Saccharomyces boulardii supplementation at a dose of 250 mg/day was not effective at reducing the incidence of death or NEC in VLBW infants, it improved feeding tolerance and reduced the risk of clinical sepsis. ©2013 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

Dual Roles of Endogenous Platelet-activating Factor Acetylhydrolase in a Murine Model of Necrotizing Enterocolitis

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Lu, Jing; Pierce, Marissa; Franklin, Andrew; Jilling, Tamas; Stafforini, Diana M.; Caplan, Michael

2010-01-01

Human preterm infants with necrotizing enterocolitis (NEC) have increased circulating and luminal levels of platelet-activating factor (PAF) and decreased serum PAF-acetylhydrolase (PAF-AH), the enzyme that inactivates PAF. Formula supplemented with recombinant PAF-AH decreases NEC in a neonatal rat model. We hypothesized that endogenous PAF-AH contributes to neonatal intestinal homeostasis, and therefore developed PAF-AH−/− mice using standard approaches to study the role of this enzyme in the neonatal NEC model. Following exposure to a well-established NEC model, intestinal tissues were evaluated for histology, pro-inflammatory cytokine mRNA synthesis, and death using standard techniques. We found that mortality rates were significantly lower in PAF-AH−/− pups compared to wild-type controls before 24 hours of life but surviving PAF-AH−/− animals were more susceptible to NEC development compared to wild-type controls. Increased NEC incidence was associated with prominent inflammation characterized by elevated intestinal mRNA expression of sPLA2, iNOS and CXCL1. In conclusion, the data support a protective role for endogenous PAF-AH in the development of NEC, and since preterm neonates have endogenous PAF-AH deficiency, this may place them at increased risk for disease. PMID:20531249

An animal model of necrotizing enterocolitis (NEC) in preterm rabbits.

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Bozeman, Andrew P; Dassinger, Melvin S; Birusingh, Rhea J; Burford, Jeffrey M; Smith, Samuel D

2013-04-01

Creation of an animal model of necrotizing enterocolitis (NEC) allowing adjustment of severity and potential recoverability is needed to study effectiveness of prevention and treatment strategies. This study describes a novel model in preterm rabbits capable of adjusting severity of NEC-like histologic changes. Rabbit pups (n = 151) were delivered by cesarean section 2 days preterm. In the treatment groups, tissue adhesive was applied to anal openings to simulate the poor intestinal function and dysmotility of preterm neonates. Pups were placed into five groups: 3INT (3 day intermittent block), 4INT (4 day intermittent block), 3COM (3 day complete block), 4COM (4 day complete block), based on differences in type of anal blockage and day of life sacrificed. The fifth group, 4CON, was comprised of a control arm (n = 28) without anal block, with sacrifice of subjects on day 4. All pups were gavage fed with formula contaminated with Enterobacter cloacae, ranitidine, and indomethacin. Following sacrifice, the intestines were harvested for pathologic evidence of NEC. A blinded pathologist graded histologic changes consistent with NEC using a grading scale 0-4 with 4 being most severe. Fifty-seven pups (57/123) (46%) in the research arm survived to sacrifice, compared to 26/28 (93%) in the control arm of the investigation, p < 0.0001. The incidence and severity of NEC-like damage increased with the duration and completeness of the anal blockage. 44/57 (77%) of survivors revealed various degrees of NEC-like damage to large and small bowel, and 3/26 (12%) exhibited early NEC-like mucosal injury in the research and control arms, respectively. This animal model produces NEC-like pathologic changes in both small and large intestine in preterm rabbits. Because incidence and severity of damage increases with duration and completeness of intestinal dysmotility, this allows future effectiveness studies for nonsurgical treatment and prevention of NEC.

A patient with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and severe hypoganglionosis associated with a novel SOX10 mutation.

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Akutsu, Yuko; Shirai, Kentaro; Takei, Akira; Goto, Yudai; Aoyama, Tomohiro; Watanabe, Akimitu; Imamura, Masatoshi; Enokizono, Takashi; Ohto, Tatsuyuki; Hori, Tetsuo; Suzuki, Keiko; Hayashi, Masaharu; Masumoto, Kouji; Inoue, Ken

2018-05-01

In this report, we present the case of a female infant with peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease (PCWH) associated with a novel frameshift mutation (c.842dupT) in exon 5, the last exon of SOX10. She had severe hypoganglionosis in the small intestine and entire colon, and suffered from frequent enterocolitis. The persistence of ganglion cells made both the diagnosis and treatment difficult in the neonatal period. She also showed hypopigmentation of the irises, hair and skin, bilateral sensorineural deafness with hypoplastic inner year, severe demyelinating neuropathy with hypotonia, and diffuse brain hypomyelination. The p.Ser282GlnfsTer12 mutation presumably escapes from nonsense-mediated decay and may generate a dominant-negative effect. We suggest that hypoganglionosis can be a variant intestinal manifestation associated with PCWH and that hypoganglionosis and aganglionosis may share the same pathoetiological mechanism mediated by SOX10 mutations. © 2018 Wiley Periodicals, Inc.

[Probiotic associations in the prevention of necrotising enterocolitis and the reduction of late-onset sepsis and neonatal mortality in preterm infants under 1,500g: A systematic review].

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Baucells, Benjamin James; Mercadal Hally, Maria; Álvarez Sánchez, Airam Tenesor; Figueras Aloy, Josep

2016-11-01

Necrotising enterocolitis (NEC) is one of the most common and serious acquired bowel diseases a premature newborn can face. This meta-analysis was performed comparing different probiotic mixtures to ascertain their benefits as a routine tool for preventing necrotising enterocolitis and reducing late-onset sepsis and mortality in premature neonates of less than 1500g. A systematic review of randomised controlled trials, between January 1980 and March 2014, on MEDLINE, the Cochrane Central Register of Controlled Trials, together with EMBASE, was carried out. Studies with infants Probiotics were found to reduce the NEC incidence (RR 0.39; 95%CI: 0.26-0.57) and mortality (RR 0.70; 95%CI: 0.52-0.93), with no difference to placebo regarding late-onset sepsis (RR 0.91; 95%CI: 0.78-1.06). Finally, when analysing the different strands, the use of a 2-probiotic combination (Lactobacillus acidophilus with Bifidobacterium bifidum) proved to be statistically significant in reducing all-cause mortality when compared to other probiotic combinations (RR 0.32; 95%CI: 0.15-0.66, NNT 20; 95%CI: 12-50). Probiotics are a beneficial tool in the prevention of NEC and mortality in preterm neonates. Moreover, the combination of 2 probiotics (Lactobacillus acidophilus with Bifidobacterium bifidum) seems to produce the greatest benefits. However, due to the differences in probiotic components and administration, it would be wise to perform a randomised controlled trial comparing different probiotic mixtures. Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

Dextran sodium sulfate (DSS induces necrotizing enterocolitis-like lesions in neonatal mice.

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Marco Ginzel

Full Text Available Necrotizing enterocolitis (NEC is an inflammatory bowel disease of preterm human newborns with yet unresolved etiology. An established neonatal murine model for NEC employs oral administration of lipopolysaccharides (LPS combined with hypoxia/hypothermia. In adult mice, feeding dextran sodium sulfate (DSS represents a well-established model for experimental inflammatory bowel disease. Here we investigated the effect of DSS administration on the neonatal murine intestine in comparison with the established NEC model.3-day-old C57BL/6J mice were either fed formula containing DSS or LPS. LPS treated animals were additionally stressed by hypoxia/hypothermia twice daily. After 72 h, mice were euthanized, their intestinal tissue harvested and analyzed by histology, qRT-PCR and flow cytometry. For comparison, adult C57BL/6J mice were fed with DSS for 8 days and examined likewise. Untreated, age matched animals served as controls.Adult mice treated with DSS exhibited colonic inflammation with significantly increased Cxcl2 mRNA expression. In contrast, tissue inflammation in neonatal mice treated with DSS or LPS plus hypoxia/hypothermia was present in colon and small intestine as well. Comparative analysis of neonatal mice revealed a significantly increased lesion size and intestinal Cxcl2 mRNA expression after DSS exposure. Whereas LPS administration mainly induced local neutrophil recruitment, DSS treated animals displayed increased monocytes/macrophages infiltration.Our study demonstrates the potential of DSS to induce NEC-like lesions accompanied by a significant humoral and cellular immune response in the small and large intestine of neonatal mice. The new model therefore represents a good alternative to LPS plus hypoxia/hypothermia administration requiring no additional physical stress.

The initial experience of antithrombin III in the management of neonates with necrotizing enterocolitis.

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St Peter, Shawn D; Little, Danny C; Calkins, Casey M; Holcomb, George W; Snyder, Charles L; Ostlie, Daniel J

2007-04-01

Necrotizing enterocolitis (NEC), the devastating enteric process of premature neonates, is marked by severe intravascular abnormalities and disseminated intravascular coagulation. Treatment to date remains historical and continues to be merely supportive without attempts to ameliorate progress within the inflammatory or coagulation cascades. Antithrombin III (ATIII) supplementation has been shown to favorably alter the process of disseminated intravascular coagulation and sepsis in adults. However, no reported use of this treatment exists in neonates. Therefore, we analyze the efficacy of our recent experience with ATIII replacement therapy in neonates with NEC. Age and diseased-matched controls with NEC were identified before the introduction of ATIII in our institution and compared against neonates with NEC undergoing ATIII replacement for diminished ATIII levels. Data collected included demographics, course of treatment parameters, and outcomes. Course of treatment parameters included hemoglobin, platelet count, prothrombin time, and partial thromboplastin time over the first 10 consecutive days of treatment. Outcome variables included packed red blood cell, platelet, fresh frozen plasma, and cryoprecipitate transfusions, as well as transfusion cost, length of stay, and survival. Over a 5-year period, 19 neonates with NEC received ATIII and were compared to 17 historical controls. Treatment hematologic profiles were not worsened in the ATIII-treated patients. The control patients received less overall transfusions and had a shorter length of stay. Antithrombin III appears to be safe in neonates with NEC, and its impact on reversing intravascular pathology in these patients warrants more thorough investigation.

Tumor Necrosis Factor-α and Apoptosis Signal-Regulating Kinase 1 Control Reactive Oxygen Species Release, Mitochondrial Autophagy and C-Jun N-Terminal Kinase/P38 Phosphorylation During Necrotizing Enterocolitis

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Naira Baregamian

2009-01-01

Full Text Available Background: Oxidative stress and inflammation may contribute to the disruption of the protective gut barrier through various mechanisms; mitochondrial dysfunction resulting from inflammatory and oxidative injury may potentially be a significant source of apoptosis during necrotizing enterocolitis (NEC. Tumor necrosis factor (TNFα is thought to generate reactive oxygen species (ROS and activate the apoptosis signal-regulating kinase 1 (ASK1-c-Jun N-terminal kinase (JNK/p38 pathway. Hence, the focus of our study was to examine the effects of TNFα/ROs on mitochondrial function, ASK1-JNK/p38 cascade activation in intestinal epithelial cells during NEC.

A Fabry-Pérot interferometer with wire-grid polarizers as beamsplitters at terahertz frequencies

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Harrison, H.; Lancaster, A. J.; Konoplev, I. V.; Doucas, G.; Aryshev, A.; Shevelev, M.; Terunuma, N.; Urakawa, J.; Huggard, P. G.

2018-03-01

The design of a compact Fabry-Pérot interferometer (FPi) and results of the experimental studies carried out using the device are presented. Our FPi uses freestanding wire-grid polarizers (WGPs) as beamsplitters and is suitable for use at terahertz (THz) frequencies. The FPi was studied at the LUCX facility, KEK, Japan, and an 8 MeV linear electron accelerator was used to generate coherent Smith-Purcell radiation. The FPi was designed to be easy to align and reposition for experiments at linear accelerator facilities. All of the components used were required to have a flat or well understood frequency response in the THz range. The performance of the FPi with WGPs was compared to that of a Michelson interferometer and the FPi is seen to perform well. The effectiveness of the beamsplitters used in the FPi is also investigated. Measurements made with the FPi using WGPs, the preferred beamsplitters, are compared to measurements made with the FPi using silicon wafers as alternative beamsplitters. The FPi performs well with both types of beamsplitter in the frequency range used (0.3-0.5 THz). The successful measurements taken with the FPi demonstrate a compact and adaptable interferometer that is capable of analyzing THz radiation over a broad frequency range. The scheme is particularly well suited for polarization studies of THz radiation produced in an accelerator environment.

Glycine reduces tissue lipid peroxidation in hypoxia-reoxygenation-induced necrotizing enterocolitis in rats

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Meyer Karine Furtado

2006-01-01

Full Text Available PURPOSE: To assess the protective effect of glycine in an experimental model of Neonatal Necrotizing Enterocolitis (NEC. METHODS: Fifty (50 neonatal Wistar rats, from a litter of six female rats and weighing 4 to 6 grams, were used. Five animals were cannibalized and the 45 remaining were distributed into three groups: the G1 normal control group (n=12; the G2 Group (n=16, of animals that underwent hypoxia-reoxygenation (HR; the G3 Group of animals (n=17 that underwent HR following a 5% intraperitoneal glycine infusion. The animals underwent hypoxia in a CO2 chamber receiving an air flow of 100% CO2 for 5 minutes and reoxygenation receiving an O2 flow at 100% for 5 minutes. One centimeter long small bowel and colon segments were prepared for histological analysis. The rest of the bowel was removed in a block and frozen at minus 80degreesC for homogenization and determination of tissue malondialdehyde (MDA. Tissue lesions were classified as Grade 0 to Grade 5, according to the level of damaged mucosa. RESULTS: The animals in Group G1 had levels of small bowel and colon lesion significantly smaller as compared to the animals in Groups G2 and G3. The G2 group had mean MDA values significantly higher than the animals in the G1 (p = .015 and G3 (p=0.021 groups. MDA values did not differ significantly (p = 0.992 for the animals in groups G1 and G3. CONCLUSION: Glycine reduces tissue MDA levels (a measurement of lipid peroxidation following HR in neonatal rats.

Cost and Cost-Effectiveness of Donor Human Milk to Prevent Necrotizing Enterocolitis: Systematic Review.

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Buckle, Abigail; Taylor, Celia

2017-11-01

Necrotizing enterocolitis (NEC) is a costly gastrointestinal disorder that mainly affects preterm and low-birth-weight infants and can lead to considerable morbidity and mortality. Mother's own milk is protective against NEC but is not always available. In such cases, donor human milk has also been shown to be protective (although to a lesser extent) compared with formula milk, but it is more expensive. This systematic review aimed at evaluating the cost of donor milk, the cost of treating NEC, and the cost-effectiveness of exclusive donor milk versus formula milk feeding to reduce the short-term health and treatment costs of NEC. We systematically searched five relevant databases to find studies with verifiable costs or charges of donor milk and/or treatment of NEC and any economic evaluations comparing exclusive donor milk with exclusive formula milk feeding. All search results were double screened. Seven studies with verifiable donor milk costs and 17 with verifiable NEC treatment costs were included. The types of cost or charge included varied considerably across studies, so quantitative synthesis was not attempted. Estimates of the incremental length of stay associated with NEC were ∼18 days for medical NEC and 50 days for surgical NEC. Two studies claimed to report economic evaluations but did not do so in practice. It is likely that donor milk provides short-term cost savings by reducing the incidence of NEC. Future studies should provide more details on cost components included and a full economic evaluation, including long-term outcomes, should be undertaken.

Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report [v1; ref status: indexed, http://f1000r.es/24q

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Dino Mijatovic

2014-03-01

Full Text Available Introduction: Hemolytic-uremic syndrome (HUS is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS, toxic megacolon with ileus, pancreatitis, central nervous system (CNS disorders and multiple organ failure (MOF. Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. In the next week of, what initially appeared as typical HUS, she developed MOF, including ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.

Clinical trial simulations in pediatric patients using realistic covariates: application to teduglutide, a glucagon-like peptide-2 analog in neonates and infants with short-bowel syndrome.

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Mouksassi, M S; Marier, J F; Cyran, J; Vinks, A A

2009-12-01

Teduglutide, a synthetic glucagon-like peptide-2 (GLP-2) analog with activity relating to the regeneration, maintenance, and repair of the intestinal epithelium, is currently being evaluated for the treatment of short-bowel syndrome (SBS), Crohn's disease, and other gastrointestinal disorders. On the basis of promising results from teduglutide studies in adults with SBS and from studies in neonatal and juvenile animal models, a pediatric multiple-dose phase I clinical study was designed to determine the safety, efficacy, and pharmacokinetics of teduglutide in pediatric patients with SBS who have undergone resection for necrotizing enterocolitis, malrotation, or intestinal atresia. This report details the application of clinical trial simulations coupled with a novel approach using generalized additive modeling for location, scale, and shape (GAMLSS) that facilitates the simulation of demographic covariates specific to the targeted patient populations. The goal was to optimize phase I dosing strategies and the likelihood of achieving target exposure and therapeutic effect.

Development of the preterm gut microbiome in twins at risk of necrotising enterocolitis and sepsis.

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Christopher J Stewart

Full Text Available The preterm gut microbiome is a complex dynamic community influenced by genetic and environmental factors and is implicated in the pathogenesis of necrotising enterocolitis (NEC and sepsis. We aimed to explore the longitudinal development of the gut microbiome in preterm twins to determine how shared environmental and genetic factors may influence temporal changes and compared this to the expressed breast milk (EBM microbiome. Stool samples (n = 173 from 27 infants (12 twin pairs and 1 triplet set and EBM (n = 18 from 4 mothers were collected longitudinally. All samples underwent PCR-DGGE (denaturing gradient gel electrophoresis analysis and a selected subset underwent 454 pyrosequencing. Stool and EBM shared a core microbiome dominated by Enterobacteriaceae, Enterococcaceae, and Staphylococcaceae. The gut microbiome showed greater similarity between siblings compared to unrelated individuals. Pyrosequencing revealed a reduction in diversity and increasing dominance of Escherichia sp. preceding NEC that was not observed in the healthy twin. Antibiotic treatment had a substantial effect on the gut microbiome, reducing Escherichia sp. and increasing other Enterobacteriaceae. This study demonstrates related preterm twins share similar gut microbiome development, even within the complex environment of neonatal intensive care. This is likely a result of shared genetic and immunomodulatory factors as well as exposure to the same maternal microbiome during birth, skin contact and exposure to EBM. Environmental factors including antibiotic exposure and feeding are additional significant determinants of community structure, regardless of host genetics.

Development of the preterm gut microbiome in twins at risk of necrotising enterocolitis and sepsis.

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Stewart, Christopher J; Marrs, Emma C L; Nelson, Andrew; Lanyon, Clare; Perry, John D; Embleton, Nicholas D; Cummings, Stephen P; Berrington, Janet E

2013-01-01

The preterm gut microbiome is a complex dynamic community influenced by genetic and environmental factors and is implicated in the pathogenesis of necrotising enterocolitis (NEC) and sepsis. We aimed to explore the longitudinal development of the gut microbiome in preterm twins to determine how shared environmental and genetic factors may influence temporal changes and compared this to the expressed breast milk (EBM) microbiome. Stool samples (n = 173) from 27 infants (12 twin pairs and 1 triplet set) and EBM (n = 18) from 4 mothers were collected longitudinally. All samples underwent PCR-DGGE (denaturing gradient gel electrophoresis) analysis and a selected subset underwent 454 pyrosequencing. Stool and EBM shared a core microbiome dominated by Enterobacteriaceae, Enterococcaceae, and Staphylococcaceae. The gut microbiome showed greater similarity between siblings compared to unrelated individuals. Pyrosequencing revealed a reduction in diversity and increasing dominance of Escherichia sp. preceding NEC that was not observed in the healthy twin. Antibiotic treatment had a substantial effect on the gut microbiome, reducing Escherichia sp. and increasing other Enterobacteriaceae. This study demonstrates related preterm twins share similar gut microbiome development, even within the complex environment of neonatal intensive care. This is likely a result of shared genetic and immunomodulatory factors as well as exposure to the same maternal microbiome during birth, skin contact and exposure to EBM. Environmental factors including antibiotic exposure and feeding are additional significant determinants of community structure, regardless of host genetics.

Valor preditivo da distensão de alças intestinais em radiografias no prognóstico de enterocolite necrosante Dilatation of bowel loops on radiological images: prognostic value in necrotizing enterocolitis

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Daniel Lahan Martins

2007-10-01

Full Text Available OBJETIVO: Avaliar o valor prognóstico da distensão de alças intestinais observada em radiografias na evolução e mortalidade de neonatos com enterocolite necrosante. MATERIAIS E MÉTODOS: Nas radiografias de abdome de 53 pacientes obtidas no momento da suspeita diagnóstica de enterocolite necrosante, foi realizada a medida do diâmetro da alça mais distendida (AD, assim como a distância entre a borda superior da primeira vértebra lombar e a borda inferior da segunda (L1-L2, a distância entre as bordas laterais dos pedículos da primeira vértebra lombar (L1, e foram estabelecidas as associações entre AD/L1-L2 e AD/L1. Esta medida foi considerada como possível determinante de potenciais complicações, intervenção cirúrgica e mortalidade. RESULTADOS: Os pacientes que necessitaram de tratamento cirúrgico, aqueles que tiveram complicações durante a evolução e aqueles que morreram da doença tiveram a relação entre AD e AD/L1-L2 maiores (p OBJECTIVE: To evaluate the prognostic value of bowel loops dilatation as a finding on radiographs in the development and mortality of neonates with necrotizing enterocolitis. MATERIALS AND METHODS: On abdominal radiographs of 53 patients for diagnostic suspicion of necrotizing enterocolitis, the major diameters of dilated bowel loops (AD were measured, as well as the distance between the upper border of the first lumbar vertebra and the lower border of the second one (L1-L2, and the distance between the lateral borders of the first lumbar vertebra (L1 pedicles, and the subsequent association between AD/L1-L2, AD/L1. This measure was considered as a possible determining factor for potential complications, surgical intervention and mortality. RESULTS: The patients who needed surgical management and who had complications during progression and died of the disease had showed increased AD and AD/L1-L2 (p < 0.05. AD/L1 values and site of the most dilated bowel loop were not different in the groups

Evaluation of a therapy for Idiopathic Chronic Enterocolitis in rhesus macaques (Macaca mulatta and linked microbial community correlates

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Joshua M. Taylor

2018-04-01

Full Text Available Idiopathic chronic enterocolitis (ICE is one of the most commonly encountered and difficult to manage diseases of captive rhesus macaques (Macaca mulatta. The etiology is not well understood, but perturbations in gut microbial communities have been implicated. Here we evaluated the effects of a 14-day course of vancomycin, neomycin, and fluconazole on animals affected with ICE, comparing treated, untreated, and healthy animals. We performed microbiome analysis on duodenal and colonic mucosal samples and feces in order to probe bacterial and/or fungal taxa potentially associated with ICE. All treated animals showed a significant and long-lasting improvement in stool consistency over time when compared to untreated and healthy controls. Microbiome analysis revealed trends associating bacterial community composition with ICE, particularly lineages of the Lactobacillaceae family. Sequencing of DNA from macaque food biscuits revealed that fungal sequences recovered from stool were dominated by yeast-derived food additives; in contrast, bacteria in stool appeared to be authentic gut residents. In conclusion, while validation in larger cohorts is needed, the treatment described here was associated with significantly improved clinical signs; results suggested possible correlates of microbiome structure with disease, though no strong associations were detected between single microbes and ICE.

Fetal cholinergic anti-inflammatory pathway and necrotizing enterocolitis: the brain-gut connection begins in utero

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Luca eGarzoni

2013-08-01

Full Text Available Necrotizing enterocolitis (NEC is an acute neonatal inflammatory disease that affects the intestine and may result in necrosis, systemic sepsis and multisystem organ failure. NEC affects 5-10% of all infants with birth weight ≤ 1500 g or gestational age less than 30 weeks. Chorioamnionitis (CA is the main manifestation of pathological inflammation in the fetus and is strongly associated with NEC. CA affects 20% of full-term pregnancies and up to 60% of preterm pregnancies and, notably, is often an occult finding. Intrauterine exposure to inflammatory stimuli may switch innate immunity cells such as macrophages to a reactive phenotype (‘priming’. Confronted with renewed inflammatory stimuli during labour or postnatally, such sensitized cells can sustain a chronic or exaggerated production of proinflammatory cytokines associated with NEC (two-hit hypothesis. Via the cholinergic anti-inflammatory pathway, a neurally mediated innate anti-inflammatory mechanism, higher levels of vagal activity are associated with lower systemic levels of proinflammatory cytokines. This effect is mediated by the α7 subunit nicotinic acetylcholine receptor (α7nAChR on macrophages. The gut is the most extensive organ innervated by the vagus nerve; it is also the primary site of innate immunity in the newborn. Here we review the mechanisms of possible neuroimmunological brain-gut interactions involved in the induction and control of antenatal intestinal inflammatory response and priming. We propose a neuroimmunological framework to 1 study the long-term effects of perinatal intestinal response to infection and 2 to uncover new targets for preventive and therapeutic intervention.

Isoelectric solubilization/precipitation as a means to recover protein isolate from striped bass (Morone saxatilis) and its physicochemical properties in a nutraceutical seafood product.

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Tahergorabi, Reza; Beamer, Sarah K; Matak, Kristen E; Jaczynski, Jacek

2012-06-13

Excessive dietary intake of Na (i.e., NaCl) contributes to hypertension, which is a major risk factor for cardiovascular disease. Normally, NaOH and HCl are used to dissolve and precipitate, respectively, fish muscle proteins in isoelectric solubilization/precipitation (ISP), therefore contributing to increased Na content in the recovered fish protein isolates (FPI). Substitution of NaOH with KOH may decrease the Na content in FPI and, thus, allow development of reduced-Na seafood products. In this study, FPI was recovered with ISP using NaOH or KOH. In order to develop a nutraceutical seafood product, the FPI was extracted with NaCl or KCl-based salt substitute and subjected to cold- or heat-gelation. In addition, standard nutraceutical additives (ω-3 fatty acids-rich oil and dietary fiber) along with titanium dioxide (TiO2) were added to FPI. Color, texture, dynamic rheology, Na and K content, and lipid oxidation of the FPI gels were compared to commercial Alaska pollock surimi gels. FPI gels had greater (p color properties (L*a*b*), and generally better textural properties when compared to surimi gels. Although the ISP-recovered FPI and surimi developed similar final gel elasticity, the proteins in FPI and surimi had different gelation pattern. A reduction (p fish for subsequent development of nutraceutical seafood products tailored for reduction of diet-driven cardiovascular disease.

Systematic review and meta-analysis of enterocolitis after one-stage transanal pull-through procedure for Hirschsprung's disease.

LENUS (Irish Health Repository)

Ruttenstock, Elke

2012-02-01

PURPOSE: The transanal one-stage pull-through procedure (TERPT) has gained worldwide popularity over open and laparoscopic-assisted one-stage techniques in children with Hirschsprung\\'s disease (HD). It offers the advantages of avoiding laparotomy, laparoscopy, scars, abdominal contamination, and adhesions. However, enterocolitis associated with Hirschsprung\\'s disease (HAEC) still remains to be a potentially life-threatening complication after pull-through operation. The reported incidence of HAEC ranges from 4.6 to 54%. This meta-analysis was designed to evaluate postoperative incidence of HAEC following TERPT procedure. METHODS: A meta-analysis of cases of TERPT reported between 1998 and 2009 was performed. Detailed information was recorded regarding intraoperative details and postoperative complications with particular emphasis on incidence of HAEC. Diagnosis of HAEC in a HD patient was based on the clinical presentation of diarrhoea, abdominal distension, and fever. RESULTS: Of the 54 published articles worldwide, 27 articles, including 899 patients were identified as reporting entirely TERPT procedure. Postoperative HAEC occurred in 92 patients (10.2%). Recurrent episodes of HAEC were reported in 18 patients (2%). Conservative treatment of HAEC was successful in 75 patients (81.5%), whereas in 17 patients (18.5%) surgical treatment was needed. CONCLUSIONS: This systematic review reveals that TERPT is a safe and less-invasive procedure with a low incidence of postoperative HAEC.

Low Microbial Diversity and Abnormal Microbial Succession Is Associated with Necrotizing Enterocolitis in Preterm Infants

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Dobbler, Priscila T.; Procianoy, Renato S.; Mai, Volker; Silveira, Rita C.; Corso, Andréa L.; Rojas, Bruna S.; Roesch, Luiz F. W.

2017-01-01

Despite increased efforts, the diverse etiologies of Necrotizing Enterocolitis (NEC) have remained largely elusive. Clinical predictors of NEC remain ill-defined and currently lack sufficient specificity. The development of a thorough understanding of initial gut microbiota colonization pattern in preterm infants might help to improve early detection or prediction of NEC and its associated morbidities. Here we compared the fecal microbiota successions, microbial diversity, abundance and structure of newborns that developed NEC with preterm controls. A 16S rRNA based microbiota analysis was conducted in a total of 132 fecal samples that included the first stool (meconium) up until the 5th week of life or NEC diagnosis from 40 preterm babies (29 controls and 11 NEC cases). A single phylotype matching closest to the Enterobacteriaceae family correlated strongly with NEC. In DNA from the sample with the greatest abundance of this phylotype additional shotgun metagenomic sequencing revealed Citrobacter koseri and Klebsiella pneumoniae as the dominating taxa. These two taxa might represent suitable microbial biomarker targets for early diagnosis of NEC. In NEC cases, we further detected lower microbial diversity and an abnormal succession of the microbial community before NEC diagnosis. Finally, we also detected a disruption in anaerobic microorganisms in the co-occurrence network of meconium samples from NEC cases. Our data suggest that a strong dominance of Citrobacter koseri and/or Klebsiella pneumoniae, low diversity, low abundance of Lactobacillus, as well as an altered microbial-network structure during the first days of life, correlate with NEC risk in preterm infants. Confirmation of these findings in other hospitals might facilitate the development of a microbiota based screening approach for early detection of NEC. PMID:29187842

Outcomes at 7 years for babies who developed neonatal necrotising enterocolitis: the ORACLE Children Study.

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Pike, Katie; Brocklehurst, Peter; Jones, David; Kenyon, Sarah; Salt, Alison; Taylor, David; Marlow, Neil

2012-09-01

Within the ORACLE Children Study Cohort, the authors have evaluated long-term consequences of the diagnosis of confirmed or suspected neonatal necrotising enterocolitis (NEC) at age of 7 years. Outcomes were assessed using a parental questionnaire, including the Health Utilities Index (HUI-3) to assess functional impairment, and specific medical and behavioural outcomes. Educational outcomes for children in England were explored using national standardised tests. Multiple logistic regression was used to explore independent associates of NEC within the cohort. The authors obtained data for 119 (77%) of 157 children following proven or suspected NEC and compared their outcomes with those of the remaining 6496 children. NEC was associated with an increase in risk of neonatal death (OR 14.6 (95% CI 10.4 to 20.6)). At 7 years, NEC conferred an increased risk of all grades of impairment. Adjusting for confounders, risks persisted for any HUI-3 defined functional impairment (adjusted OR 1.55 (1.05, 2.29)), particularly mild impairment (adjusted OR 1.61 (1.03, 2.53)) both in all NEC children and in those with proven NEC, which appeared to be independent. No behavioural or educational associations were confirmed. Following NEC, children were more likely to suffer bowel problems than non-NEC children (adjusted OR 3.96 (2.06, 7.61)). The ORACLE Children Study provided opportunity for the largest evaluation of school age outcome following neonatal NEC and demonstrates significant long-term consequences of both gut function (presence of stoma, admission for bowel problems and continuing medical care for gut-related problems) and motor, sensory and cognitive outcomes as measured using HUI-3.

Platelet-activating factor induces TLR4 expression in intestinal epithelial cells: implication for the pathogenesis of necrotizing enterocolitis.

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Antoine Soliman

Full Text Available Necrotizing enterocolitis (NEC is a leading cause of morbidity and mortality in neonatal intensive care units, however its pathogenesis is not completely understood. We have previously shown that platelet activating factor (PAF, bacteria and TLR4 are all important factors in the development of NEC. Given that Toll-like receptors (TLRs are expressed at low levels in enterocytes of the mature gastrointestinal tract, but were shown to be aberrantly over-expressed in enterocytes in experimental NEC, we examined the regulation of TLR4 expression and signaling by PAF in intestinal epithelial cells using human and mouse in vitro cell lines, and the ex vivo rat intestinal loop model. In intestinal epithelial cell (IEC lines, PAF stimulation yielded upregulation of both TLR4 mRNA and protein expression and led to increased IL-8 secretion following stimulation with LPS (in an otherwise LPS minimally responsive cell line. PAF stimulation resulted in increased human TLR4 promoter activation in a dose dependent manner. Western blotting and immunohistochemical analysis showed PAF induced STAT3 phosphorylation and nuclear translocation in IEC, and PAF-induced TLR4 expression was inhibited by STAT3 and NFκB Inhibitors. Our findings provide evidence for a mechanism by which PAF augments inflammation in the intestinal epithelium through abnormal TLR4 upregulation, thereby contributing to the intestinal injury of NEC.

Eficácia dos probióticos na profilaxia de enterocolite necrosante em recém-nascidos prematuros: revisão sistemática e meta-análise Effectiveness of probiotics in the prophylaxis of necrotizing enterocolitis in preterm neonates: a systematic review and meta-analysis

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Wanderley Marques Bernardo

2013-02-01

Full Text Available OBJETIVO: Elucidar os benefícios do uso de probióticos na prevenção de enterocolite necrosante (ECN e de suas complicações em recém-nascidos prematuros. MÉTODO: Revisão sistemática de ensaios clínicos randomizados, que incluiu pesquisas efetuadas em três bases de dados (MEDLINE, EMBASE e LILACS, utilizando a combinação dos termos (necrotizing enterocolitis AND (probiotics. RESULTADOS : Foram incluídos 11 ensaios clínicos randomizados, totalizando 2.887 pacientes, sendo 1.431 no grupo Probiótico e 1.456 no grupo Controle. Houve redução na incidência de ECN (NNT = 25, de morte global (NNT = 34 e sepse neonatal (NNT = 34 no grupo Probiótico em relação ao grupo Controle. Pacientes alimentados com suplementação de probióticos tiveram tempo de reintrodução alimentar (p OBJECTIVE: To elucidate the benefits of using probiotics in the prevention of necrotizing enterocolitis (NEC and its complications in preterm newborns. METHOD: This was a systematic review of randomized controlled trials, which included studies retrieved from three databases (MEDLINE, Embase, and LILACS, using a combination of the terms (necrotizing enterocolitis AND (probiotics. RESULTS: 11 randomized trials were included, totaling 2,887 patients, 1,431 in the probiotic group and 1,456 in the control group. There was a reduction in the incidence of NEC (NNT = 25, overall death (NNT = 34, and neonatal sepsis (NNT = 34 in the probiotic group compared to the control group. Patients that received probiotic supplementation had lower food reintroduction time (p < 0.001 and hospitalization time (p < 0.001 when compared to those not receiving probiotics. There was no difference in mortality caused by NEC. CONCLUSION: In premature newborns, the use of probiotics is effective as a prophylaxis for NEC and its complications.

Gram negative bacteria are associated with the early stages of necrotizing enterocolitis.

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Erica M Carlisle

Full Text Available INTRODUCTION: Necrotizing enterocolitis (NEC affects 5-10% of infants born weighing less than 1500 g. Most models of NEC recapitulate late-stage disease with gut necrosis and elevated inflammatory mediators. Evaluation of NEC at earlier, less lethal stages of disease will allow investigation of initial disease triggers and may advance our understanding of temporal relationships between factors implicated in NEC pathogenesis. In this manuscript, we describe our investigation of early NEC and test the hypothesis that bacteria and inflammatory mediators differ between animals with early NEC and disease free animals. METHODS: On DOL7 C3HeB/FeJ pups were fed liquid formula with 1×10(4 Streptococcus thoraltensis, Serratia marcescens, and Pseudomonas aeruginosa every 3 h. To initiate NEC, pups underwent asphyxia (100% N(2 for 90 s and hypothermia (4°C for 10 min after feeding. Pups were euthanized at 72 h. Intestines were collected for histologic NEC scoring and DNA/RNA extraction. Bacterial populations were identified by 16S rRNA pyrosequencing and principal component analysis (PCA. RNA isolates underwent QRT-PCR for Toll-like Receptor 4 (TLR4 and inducible nitric oxide synthase (iNOS. RESULTS: Despite histologic, intestinal damage in mice with NEC, no gross necrosis was observed suggesting early disease. QRT-PCR yielded no difference between groups in TLR4 or iNOS mRNA levels. PCA demonstrated relative clustering of microbial communities based on presence or absence of NEC. 16S pyrosequencing demonstrated similar phyla between groups (Firmicutes and Proteobacteria predominated in all animals. However, the colonic microbiota of animals with NEC had more Citrobacter (p<0.01, Klebsiella (p<0.05, and Tatumella (p<0.05, while that of animals without NEC had more Streptococcus (p<0.01 and Enterococcus (p<0.01. CONCLUSION: Citrobacter, Klebsiella, and Tatumella are associated with NEC. Differential colonic bacteria were identified despite the lack of

Antenatal treatment with corticosteroids for preterm neonates: impact on the incidence of respiratory distress syndrome and intra-hospital mortality

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Joice Fabíola Meneguel

Full Text Available CONTEXT: Although the benefits of antenatal corticosteroids have been widely demonstrated in other countries, there are few studies among Brazilian newborn infants. OBJECTIVE: To evaluate the effectiveness of antenatal corticosteroids on the incidence of respiratory distress syndrome and intra-hospital mortality among neonates with a gestational age of less than 34 weeks. TYPE OF STUDY: Cross-sectional. SETTING: A tertiary-care hospital. PARTICIPANTS: Neonates exposed to any dose of antenatal corticosteroids for fetal maturation up to 7 days before delivery, and newborns paired by sex, birth weight, gestational age and time of birth that were not exposed to antenatal corticosteroids. The sample obtained consisted of 205 exposed newborns, 205 non-exposed and 39 newborns exposed to antenatal corticosteroids for whom it was not possible to find an unexposed pair. PROCEDURES: Analysis of maternal and newborn records. MAIN MEASUREMENTS: The primary clinical outcomes for the two groups were compared: the incidence of respiratory distress syndrome and intra-hospital mortality; as well as secondary outcomes related to neonatal morbidity. RESULTS: Antenatal corticosteroids reduced the occurrence of respiratory distress syndrome (OR: 0.33; 95% CI: 0.21-0.51 and the protective effect persisted when adjusted for weight, gestational age and the presence of asphyxia (adjusted OR: 0.27; 95% CI: 0.17-0.43. The protective effect could also be detected through the reduction in the need for and number of doses of exogenous surfactant utilized and the number of days of mechanical ventilation needed for the newborns exposed to antenatal corticosteroids. Their use also reduced the occurrence of intra-hospital deaths (OR: 0.51: 95% CI: 0.38-0.82. However, when adjusted for weight, gestational age, presence of prenatal asphyxia, respiratory distress syndrome, necrotizing enterocolitis and use of mechanical ventilation, the antenatal corticosteroids did not maintain the

28 CFR 345.83 - Job safety training.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Job safety training. 345.83 Section 345... INDUSTRIES (FPI) INMATE WORK PROGRAMS FPI Inmate Training and Scholarship Programs § 345.83 Job safety training. FPI provides inmates with regular job safety training which is developed and scheduled in...

DYNSUB: A high fidelity coupled code system for the evaluation of local safety parameters – Part II: Comparison of different temporal schemes

International Nuclear Information System (INIS)

Gomez-Torres, Armando Miguel; Sanchez-Espinoza, Victor Hugo; Ivanov, Kostadin; Macian-Juan, Rafael

2012-01-01

Highlights: ► A fixed point iteration (FPI) is implemented in DYNSUB. ► Comparisons between the explicit scheme and the FPI are done. ► The FPI scheme allows moving from one time step to the other with converged solution. ► FPI allows the use of larger time steps without compromising the accuracy of results. ► FPI results are promising and represent an option in order to optimize calculations. -- Abstract: DYNSUB is a novel two-way pin-based coupling of the simplified transport (SP 3 ) version of DYN3D with the subchannel code SUBCHANFLOW. The new coupled code system allows for a more realistic description of the core behaviour under steady state and transients conditions, and has been widely described in Part I of this paper. Additionally to the explicit coupling developed and described in Part I, a nested loop iteration or fixed point iteration (FPI) is implemented in DYNSUB. A FPI is not an implicit scheme but approximates it by adding an iteration loop to the current explicit scheme. The advantage of the method is that it allows the use of larger time steps; however the nested loop iteration could take much more time in getting a converged solution that could be less efficient than the explicit scheme with small time steps. A comparison of the two temporal schemes is performed. The results using FPI are very promising and represent a very good option in order to optimize computational times without losing accuracy. However it is also shown that a FPI scheme can produce inaccurate results if the time step is not chosen in agreement with the analyzed transient.

Uso de fatores de crescimento epidérmico e estimulador de colônias de granulócitos na prevenção e tratamento da enterocolite necrosante no recém-nascido Use of epidermic and granulocyte-colony stimulating growth factors in the prevention and treatment of necrotizing enterocolitis of the newborn

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Dáfne Cardoso B. da Silva

2008-06-01

Full Text Available OBJETIVO: Revisar os conhecimentos existentes em relação ao uso de fatores de crescimento epidérmico e estimulador de colônias de granulócitos na prevenção e/ou no tratamento da enterocolite necrosante (ECN durante o período neonatal. FONTES DE DADOS: Revisão da literatura, nas bases de dados Medline, Lilacs, SciELO e PubMed, utilizando os unitermos "recém-nascidos", "enterocolite" e "fatores de crescimento", no período de 2003 a 2007. Nesta busca, 49 artigos foram encontrados, sendo 17 pertinentes ao tema. Também foram utilizados outros artigos, independente do ano de publicação, relacionados a aspectos definidores da ECN no recém-nascido. SÍNTESE DOS DADOS: A ECN continua sendo responsável por uma elevada morbimortalidade neonatal. Os mecanismos fisiopatológicos vêm sendo elucidados e, a partir deles, são discutidas novas terapias, como o uso de fatores de crescimento, destacando-se o fator de crescimento epidérmico e o fator estimulador de colônias de granulócitos. CONCLUSÕES: O uso de fatores de crescimento no tratamento e prevenção da ECN neonatal parece promissor. É necessário maior número de ensaios clínicos para comprovar sua eficácia e segurança. Enquanto isso, a melhor prática médica continua sendo a prevenção da doença.OBJECTIVE: To review the literature regarding the use of hematopoietic and epidermic growth factors for prevention or treatment of neonatal necrotizing enterocolitis (NEC. DATA SOURCES: Literature review of Medline, Lilacs, SciELO and Pubmed databases, using the key-words "newborn", "enterocolitis" and "growth factors", from 2003 to 2007. Fourty-nine papers were retrieved, but only 17 related to the subject. Other studies that described some clinical aspects of enterocolitis were also included, regardless of the year of publication. DATA SYNTHESIS: Necrotizing and enterocolitis has been an important cause of morbidity and mortality in the neonatal period. As the knowledge about the

Using a mathematical model to analyze the role of probiotics and inflammation in necrotizing enterocolitis.

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Julia C Arciero

2010-04-01

Full Text Available Necrotizing enterocolitis (NEC is a severe disease of the gastrointestinal tract of pre-term babies and is thought to be related to the physiological immaturity of the intestine and altered levels of normal flora in the gut. Understanding the factors that contribute to the pathology of NEC may lead to the development of treatment strategies aimed at re-establishing the integrity of the epithelial wall and preventing the propagation of inflammation in NEC. Several studies have shown a reduced incidence and severity of NEC in neonates treated with probiotics (beneficial bacteria species.The objective of this study is to use a mathematical model to predict the conditions under which probiotics may be successful in promoting the health of infants suffering from NEC. An ordinary differential equation model is developed that tracks the populations of pathogenic and probiotic bacteria in the intestinal lumen and in the blood/tissue region. The permeability of the intestinal epithelial layer is treated as a variable, and the role of the inflammatory response is included. The model predicts that in the presence of probiotics health is restored in many cases that would have been otherwise pathogenic. The timing of probiotic administration is also shown to determine whether or not health is restored. Finally, the model predicts that probiotics may be harmful to the NEC patient under very specific conditions, perhaps explaining the detrimental effects of probiotics observed in some clinical studies.The reduced, experimentally motivated mathematical model that we have developed suggests how a certain general set of characteristics of probiotics can lead to beneficial or detrimental outcomes for infants suffering from NEC, depending on the influences of probiotics on defined features of the inflammatory response.

Abnormal Doppler flow velocimetry in the growth restricted foetus as a predictor for necrotising enterocolitis.

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Bhatt A

2002-07-01

Full Text Available BACKGROUND: Obstetric decision- making for the growth restricted foetus has to take into consideration the benefits and risks of waiting for pulmonary maturity and continued exposure to hostile intra-uterine environment. Necrotising Enterocolitis (NEC results from continued exposure to hostile environment and is an important cause of poor neonatal outcome. AIMS: To evaluate the predictive value of abnormal Doppler flow velocimetry of the foetal umbilical artery for NEC and neonatal mortality. SETTINGS AND DESIGN: A retrospective study carried out at a tertiary care centre for obstetric and neonatal care. MATERIALS AND METHOD: Seventy-seven neonates with birth weight less than 2000 gm, born over a period of 18 months were studied. These pregnancies were identified as having growth abnormalities of the foetus. Besides other tests of foetal well-being, they were also subjected to Doppler flow velocimetry of the foeto-placental vasculature. Obstetric outcome was evaluated with reference to period of gestation and route of delivery. The neonatal outcome was reviewed with reference to birth weight, Apgar scores and evidence of NEC. STATISTICAL ANALYSIS USED: Chi square test. RESULTS: In the group of patients with Absent or Reverse End Diastolic Frequencies (A/R EDF in the umbilical arteries, positive predictive value for NEC was 52.6%, (RR 30.2; OR 264. The mortality from NEC was 50%. When umbilical artery velocimetry did not show A/REDF, there were no cases of NEC or mortality. Abnormal umbilical or uterine artery flow increased the rate of caesarean section to 62.5% as compared to 17.6% in cases where umbilical artery flow was normal. CONCLUSION: In antenatally identified pregnancies at risk for foetal growth restriction, abnormal Doppler velocimetry in the form of A/REDF in the umbilical arteries is a useful guide to predict NEC and mortality in the early neonatal period.

Shelf-life extension of refrigerated sea bass slices wrapped with fish protein isolate/fish skin gelatin-ZnO nanocomposite film incorporated with basil leaf essential oil.

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Arfat, Yasir Ali; Benjakul, Soottawat; Vongkamjan, Kitiya; Sumpavapol, Punnanee; Yarnpakdee, Suthasinee

2015-10-01

Microbiological, chemical and sensory changes of sea bass slices wrapped with fish protein isolate (FPI)/fish skin gelatin (FSG) films incorporated with 3 % ZnO nanoparticles (ZnONP) (w/w, based on protein content) and 100 % basil leaf essential oil (BEO) (w/w, based on protein content) during storage of 12 days at 4 °C were investigated. Sea bass slices wrapped with FPI/FSG-ZnONP-BEO film had the lowest growth of psychrophilic bacteria, lactic acid bacteria and spoilage microorganisms including Pseudomonas , H2S-producing bacteria and Enterobacteriaceae throughout storage of 12 days in comparison with those wrapped with FPI/FSG-BEO, FPI/FSG-ZnONP, FPI/FSG film, polypropylene film (PP film) and the control (without wrapping), respectively (P < 0.05). Lowered increases in pH, total volatile base, peroxide value and TBARS value were found in FPI/FSG-ZnO-BEO film wrapped samples, compared with others (P < 0.05). Sensory evaluation revealed that shelf-life of sea bass slices was longest for samples wrapped with FPI/FSG-ZnONP-BEO film (12 days), as compared to the control (6 days) (P < 0.05).

Enteral intestinal alkaline phosphatase administration in newborns decreases iNOS expression in a neonatal necrotizing enterocolitis rat model.

Science.gov (United States)

Rentea, Rebecca M; Liedel, Jennifer L; Fredrich, Katherine; Pritchard, Kirkwood; Oldham, Keith T; Simpson, Pippa M; Gourlay, David M

2013-01-01

To determine if intestinal alkaline phosphatase (IAP) decreases intestinal injury resulting from experimentally induced necrotizing enterocolitis (NEC). We hypothesized that IAP administration prevents the initial development of NEC related intestinal inflammation. Pre- and full-term newborn Sprague-Dawley rat pups were sacrificed on day 1 of life. Pre-term pups were exposed to intermittent hypoxia and formula containing LPS to induce NEC. Select NEC pups were given 40, 4 or 0.4 units/kg of bovine IAP (NEC+IAP40u, IAP4u or IAP0.4u) enterally, once daily. Ileal sections were evaluated by real-time PCR (qRT-PCR) for IAP, iNOS, IL-1β, IL-6, and TNF-α mRNA and immunofluorescence for 3-nitrotyrosine (3-NT). Experimentally induced NEC decreased IAP mRNA expression by 66% (p ≤ 0.001). IAP supplementation increased IAP mRNA expression to control. Supplemental enteral IAP decreased nitrosative stress as measured by iNOS mRNA expression and 3-NT staining in the NEC stressed pups (p ≤ 0.01), as well as decreased intestinal TNF-α mRNA expression. In addition, IAP decreased LSP translocation into the serum in the treated pups. We conclude that enterally administered IAP prevents NEC-related intestinal injury and inflammation. Enteral IAP may prove a useful strategy in the prevention of NEC in preterm neonates. Copyright © 2013 Elsevier Inc. All rights reserved.

Characteristic of sausages as influenced by partial replacement of pork back-fat using pre-emulsified soybean oil stabilized by fish proteins isolate

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Nopparat Cheetangdee

2017-08-01

Full Text Available Substitution of animal fat with oils rich in n-3 is a feasible way to improve the nutritive value of comminuted meat product. The effect on the characteristics of sausages was investigated of partial replacement of porcine fat with soybean oil (SBO using a pre-emulsification technique. Fish protein isolate (FPI produced from yellow stripe trevally (Selaroides leptolepis was used as an emulsifier to prepare pre-emulsified SBO (preSBO, and its concentration effect (1%, 2% and 3%, w/v was observed in comparison with soy protein isolate (SPI. Substitution of porcine fat using preSBO enhanced the product stability. SPI exhibited better emulsifying ability than FPI. However, FPI was more effective at reinforcing the protein matrix of the sausages than SPI, as suggested by a lowered cooking loss and the restored textural attributes of the sausages formulated with FPI stabilized preSBO. The effective concentration of FPI to improve the product stability was 2%. This work suggested that FPI was promising in the preparation of emulsified meat products.

Variability in Antibiotic Regimens for Surgical Necrotizing Enterocolitis Highlights the Need for New Guidelines.

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Blackwood, Brian P; Hunter, Catherine J; Grabowski, Julia

Necrotizing enterocolitis or NEC is the most common gastrointestinal emergency in the newborn. The etiology of NEC remains unknown, and treatment consists of antibiotic therapy and supportive care with the addition of surgical intervention as necessary. Unlike most surgical diseases, clear guidelines for the type and duration of peri-operative antibiotic therapy have not been established. Our aim was to review the antibiotic regimen(s) applied to surgical patients with NEC within a single neonatal intensive care unit (NICU) and to evaluate outcomes and help develop guidelines for antibiotic administration in this patient population. A single-center retrospective review was performed of all patients who underwent surgical intervention for NEC from August 1, 2005 through August 1, 2015. Relevant data were extracted including gestational age, age at diagnosis, gender, pre-operative antibiotic treatment, post-operative antibiotic treatment, development of stricture, and mortality. Patients were excluded if there was incomplete data documentation. A total of 90 patients were identified who met inclusion criteria. There were 56 male patients and 34 female patients. The average gestational age was 30 5/7 wks and average age of diagnosis 16.7 d. A total of 22 different pre-operative antibiotic regimens were identified with an average duration of 10.6 d. The most common pre-operative regimen was ampicillin, gentamicin, and metronidazole for 14 d. A total of 15 different post-operative antibiotic regimens were identified with an average duration of 6.6 d. The most common post-operative regimen was ampicillin, gentamicin, and metronidazole for two days. There were 26 strictures and 15 deaths. No regimen or duration proved superior. We found that there is a high degree of variability in the antibiotic regimen for the treatment of NEC, even within a single NICU, with no regimen appearing superior over another. As data emerge that demonstrate the adverse effects of

Understanding Bartter syndrome and Gitelman syndrome.

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Fremont, Oliver T; Chan, James C M

2012-02-01

We aim to review the clinical features of two renal tubular disorders characterized by sodium and potassium wasting: Bartter syndrome and Gitelman syndrome. Selected key references concerning these syndromes were analyzed, together with a PubMed search of the literature from 2000 to 2011. The clinical features common to both conditions and those which are distinct to each syndrome were presented. The new findings on the genetics of the five types of Bartter syndrome and the discrete mutations in Gitelman syndrome were reviewed, together with the diagnostic workup and treatment for each condition. Patients with Bartter syndrome types 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. They present with symptoms, often quite severe in the neonatal period. Patients with classic Bartter syndrome type 3 present later in life and may be sporadically asymptomatic or mildly symptomatic. The severe, steady-state hypokalemia in Bartter syndrome and Gitelman syndrome may abruptly become life-threatening under certain aggravating conditions. Clinicians need to be cognizant of such renal tubular disorders, and promptly treat at-risk patients.

28 CFR 345.60 - Training pay.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Training pay. 345.60 Section 345.60... (FPI) INMATE WORK PROGRAMS Inmate Pay and Benefits § 345.60 Training pay. Inmates directed by the SOI to take a particular type of training in connection with a FPI job are to receive FPI pay if the...

Investigation of Ionospheric Disturbances Using Radio and Optical Observations in South-East Asia -- The Initial Results of the ASI and FPI Observations in Chiang Mai, Thailand

Science.gov (United States)

Kubota, M.; Nagatsuma, T.; Otsuka, Y.; Shiokawa, K.; Komonjinda, S.; Komolmis, T.; Somboon, E.; Tsugawa, T.; Maruyama, T.; Murata, K. T.

2010-12-01

For the purpose of monitoring and forecasting equatorial ionospheric disturbances, SEALION (SouthEast Asia Low-latitude IOnospheric Network) has been developed since 2003 as a cooperation project by National Institute of Information and Communications Technology (NICT), King Mongkut's Institute of Technology Ladkrabang (KMITL) in Thailand, Chiang Mai University (CMU) in Thailand, National Institute of Aeronautics and Space (LAPAN) in Indonesia, Hanoi Institute of Geophysics (HIG), Vietnamese Academy of Science and Technology in Vietnam, Center for Space Science and Applied Research (CSSAR), Chinese Academy of Sciences in China, Kyoto University in Japan, and Solar-Terrestrial Environment Laboratory (STEL), Nagoya University in Japan. SEALION consists of five ionosondes, four GPS receivers, two GPS scintillation monitors, and a magnetometer. As a part of this project, we newly installed an all-sky imager (ASI) and a Fabry-Perot Interferometer (FPI) at Sirindhorn observatory in Chiang Mai (18.8N, 98.9E, Dip lat. 13.1), Thailand. This site is located near conjugate to EAR site in Kototabang, Indonesia. One of main targets of the ASI observation is the large-scale wave structure (LSWS) with wavelengths of 100-1000 km. The LSWS is thought to be connected to the generation mechanism of equatorial plasma bubbles (EPB). The optical observations in Chiang Mai started in February 2010, and we have detected several ionospheric disturbance events with these instruments In this paper, we will show the initial results of the optical observations from Sirindhorn observatory, and discuss the features of ionospheric disturbances in Southeast Asia.

The Roles of Bacteria and TLR4 in Rat and Murine Models of Necrotizing Enterocolitis1

Science.gov (United States)

Jilling, Tamas; Simon, Dyan; Lu, Jing; Meng, Fan Jing; Li, Dan; Schy, Robert; Thomson, Richard B.; Soliman, Antoine; Arditi, Moshe; Caplan, Michael S.

2009-01-01

Bacteria are thought to contribute to the pathogenesis of necrotizing enterocolitis (NEC), but it is unknown whether their interaction with the epithelium can participate in the initiation of mucosal injury or they can act only following translocation across a damaged intestinal barrier. Our aims were to determine whether bacteria and intestinal epithelial TLR4 play roles in a well-established neonatal rat model and a novel neonatal murine model of NEC. Neonatal rats, C57BL/6J, C3HeB/FeJ (TLR4 wild type), and C3H/HeJ (TLR4 mutant) mice were delivered by Cesarean section and were subjected to formula feeding and cold asphyxia stress or were delivered naturally and were mother-fed. NEC incidence was evaluated by histological scoring, and gene expression was quantified using quantitative real-time PCR from cDNA generated from intestinal total RNA or from RNA obtained by laser capture microdissection. Spontaneous feeding catheter colonization or supplementation of cultured bacterial isolates to formula increased the incidence of experimental NEC. During the first 72 h of life, i.e., the time frame of NEC development in this model, intestinal TLR4 mRNA gradually decreases in mother-fed but increases in formula feeding and cold asphyxia stress, correlating with induced inducible NO synthase. TLR4, inducible NO synthase, and inflammatory cytokine induction occurred in the intestinal epithelium but not in the submucosa. NEC incidence was diminished in C3H/HeJ mice, compared with C3HeB/FeJ mice. In summary, bacteria and TLR4 play significant roles in experimental NEC, likely via an interaction of intraluminal bacteria and aberrantly overexpressed TLR4 in enterocytes. PMID:16920968

Nutritional strategies and gut microbiota composition as risk factors for necrotizing enterocolitis in very-preterm infants.

Science.gov (United States)

Rozé, Jean-Christophe; Ancel, Pierre-Yves; Lepage, Patricia; Martin-Marchand, Laetitia; Al Nabhani, Ziad; Delannoy, Johanne; Picaud, Jean-Charles; Lapillonne, Alexandre; Aires, Julio; Durox, Mélanie; Darmaun, Dominique; Neu, Josef; Butel, Marie-José

2017-09-01

Background: The pathophysiology of necrotizing enterocolitis (NEC) remains poorly understood. Objective: We assessed the relation between feeding strategies, intestinal microbiota composition, and the development of NEC. Design: We performed a prospective nationwide population-based study, EPIPAGE 2 (Etude Epidémiologique sur les Petits Ages Gestationnels), including preterm infants born at Etude Epidémiologique de la flore), in 20 of the 64 NICUs, analyzed the intestinal microbiota by culture and 16S ribosomal RNA gene sequencing. Results: Among the 3161 enrolled preterm infants, 106 (3.4%; 95% CI: 2.8%, 4.0%) developed NEC. Individual characteristics were significantly associated with NEC. Slower and intermediate rates of progression of enteral feeding strategies were associated with a higher risk of NEC, with an adjusted OR of 2.3 (95% CI: 1.2, 4.5; P = 0.01) and 2.0 (95% CI: 1.1, 3.5; P = 0.02), respectively. Less favorable and intermediate direct-breastfeeding policies were associated with higher NEC risk as well, with an adjusted OR of 2.5 (95% CI: 1.1, 5.8; P = 0.03) and 2.3 (95% CI: 1.1, 4.8; P = 0.02), respectively. Microbiota analysis performed in 16 cases and 78 controls showed an association between Clostridium neonatale and Staphylococcus aureus with NEC ( P = 0.001 and P = 0.002). Conclusions: A slow rate of progression of enteral feeding and a less favorable direct-breastfeeding policy are associated with an increased risk of developing NEC. For a given level of risk assessed by propensity score, colonization by C. neonatale and/or S. aureus is significantly associated with NEC. This trial (EPIFLORE study) was registered at clinicaltrials.gov as NCT01127698. © 2017 American Society for Nutrition.

[Maxillofacial and dental abnormalities in some multiple abnormality syndromes. "Cri du chat" syndrome, Wilms' tumor-aniridia syndrome; Sotos syndrome; Goldenhar syndrome].

Science.gov (United States)

Berio, A; Trucchi, R; Meliota, M

1992-05-01

The paper describes the maxillo-facial and dental anomalies observed in some chromosome and non-chromosome poly-malformative syndromes ("Cri du chat" syndrome; Wilms' tumour; Sotos' syndrome; Goldenhar's syndrome). The Authors emphasise the possibility of diagnosing these multiple deformity syndromes from maxillo-facial alterations in early infancy; anomalous tooth position and structure cal also be successfully treated immediately after the first appearance of teeth. This is a particularly promising field of pediatrics and preventive pediatric medicine.

Personality, depression, and premorbid lifestyle in twin pairs discordant for Parkinson's disease

Science.gov (United States)

Heberlein, I.; Ludin, H.; Scholz, J.; Vieregge, P.

1998-01-01

Present personality traits (Freiburg personality inventory, FPI-R), depression (von Zerssen's depression scale), and self assessed state of health were evaluated in 15 twin pairs (six monozygotic and nine dizygotic; mean age 62.5 years) discordant for idiopathic Parkinson's disease and in 17 unrelated healthy control subjects. The twins had additional questionnaire based interviews on premorbid lifestyle.
For disability, twins with Parkinson's disease scored lower on FPI-R than controls in "achievement orientation" and "extraversion", higher in "inhibitedness", "somatic complaints", and "emotionality". They scored higher for depression and for state of health than unaffected twins and controls. For zygosity, monozygotic twins scored lower than dizygotic twins in "achievement orientation", "aggressiveness", and "strain". Monozygotic twins had less "achievement orientation" and "extraversion" and more "somatic complaints" than controls. Monozygotic twins had a lower within pair difference than dizygotic twins in "social orientation". During premorbid times the affected twin with later Parkinson's disease was estimated to have been "less often the leader" in the twin pair.
Although small in sample size, this twin study indicates a genetic impact for some personality features beyond the Parkinson's disease motor syndrome.

 PMID:9489545

Roles and Experiences of Parents in Necrotizing Enterocolitis: An International Survey of Parental Perspectives of Communication in the NICU.

Science.gov (United States)

Gadepalli, Samir K; Canvasser, Jennifer; Eskenazi, Yael; Quinn, Megan; Kim, Jae H; Gephart, Sheila M

2017-12-01

Although partnering with parents is important to improving neonatal outcomes, no studies have investigated what parents are taught, remember, or experience when their child is afflicted with necrotizing enterocolitis (NEC). To characterize parental perceptions of communication and support they were given about NEC. An online survey was developed, reviewed for face validity, and then administered to parents whose child had experienced NEC. Quantitative data were analyzed using descriptive statistics, and qualitative data were analyzed using a qualitative descriptive approach. Parents (N = 110) wanted to know the risk factors and warning signs for NEC and wanted to be told as soon as their child was admitted to the neonatal intensive care unit (NICU). Information provided before diagnosis was felt to be poor by the majority of families, with only 32% feeling satisfied or very satisfied. No parent wrote that they were "scared" by information provided to them about NEC; in fact, parents were dissatisfied when they received "sugar-coated" information. Engaged parents were significantly more satisfied than those who were not informed, had their concerns and suggestions dismissed, or who had to advocate for their baby against clinician opposition (eg, activating the chain of command). Areas for quality improvement include better communication and collaboration with parents through early engagement in NEC prevention using modalities beyond verbal instruction. More research is needed on how best to engage parents, especially to engage in prevention, and how doing so affects satisfaction and outcomes.

Current Knowledge of Necrotizing Enterocolitis in Preterm Infants and the Impact of Different Types of Enteral Nutrition Products.

Science.gov (United States)

Shulhan, Jocelyn; Dicken, Bryan; Hartling, Lisa; Larsen, Bodil Mk

2017-01-01

Preterm infants are extremely vulnerable to a range of morbidities and mortality. Underdeveloped cardiac, respiratory, gastrointestinal, and immune systems in the preterm period increase the risk of necrotizing enterocolitis (NEC), a serious disease of the gut. NEC affects 5-12% of very-low birth-weight infants, leads to surgery in 20-40% of cases, and is fatal in 25-50% of cases. There are multiple factors that may contribute to NEC, but the exact cause is not yet fully understood. Severe cases can result in intestinal resection or death, and the health care costs average >$300,000/infant when surgical management is required. Different types of nutrition may affect the onset or progression of NEC. Several studies have indicated that bovine milk-based infant formulas lead to a higher incidence of NEC in preterm infants than does human milk (HM). However, it is not clear why HM is linked to a lower incidence of NEC or why some infants fed an exclusively HM diet still develop NEC. An area that has not been thoroughly explored is the use of semielemental or elemental formulas. These specialty formulas are easy to digest and absorb in the gut and may be an effective nutritional intervention for reducing the risk of NEC. This review summarizes what is known about the factors that contribute to the onset and progression of NEC, discusses its health care cost implications, and explores the impact that different formulas and HM have on this disease. © 2017 American Society for Nutrition.

Marfan syndrome masked by Down syndrome?

NARCIS (Netherlands)

Vis, J.C.; Engelen, K. van; Timmermans, J.; Hamel, B.C.J.; Mulder, B.J.

2009-01-01

Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome.

The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.

Science.gov (United States)

Rice, Lauren J; Gray, Kylie M; Howlin, Patricia; Taffe, John; Tonge, Bruce J; Einfeld, Stewart L

2015-06-01

The aim of this study was to investigate the developmental trajectories of verbal aggression, physical aggression, and temper tantrums in four genetic syndrome groups. Participants were part of the Australian Child to Adult Development Study (ACAD), which collected information from a cohort of individuals with an intellectual disability at five time points over 18 years. Data were examined from a total of 248 people with one of the four following syndromes: Down syndrome, Fragile X syndrome, Prader-Willi syndrome, or Williams syndrome. Changes in behaviors were measured using validated items from the Developmental Behavior Checklist (DBC). The results indicate that, while verbal aggression shows no evidence of diminishing with age, physical aggression, and temper tantrums decline with age before 19 years for people with Down syndrome, Fragile X syndrome, and William syndrome; and after 19 years for people with Prader-Willi syndrome. These findings offer a somewhat more optimistic outlook for people with an intellectual disability than has previously been suggested. Research is needed to investigate the mechanisms predisposing people with PWS to persistence of temper tantrums and physical aggression into adulthood. © 2015 Wiley Periodicals, Inc.

Animal models of gastrointestinal and liver diseases. Animal models of infant short bowel syndrome

DEFF Research Database (Denmark)

Sangild, Per Torp; Ney, Denise M; Sigalet, David L

2014-01-01

enterocolitis, atresia, gastroschisis, volvulus and aganglionosis. Patient outcomes have improved, but there is a need to develop new therapies for SBS and to understand intestinal adaptation after different diseases, resection types, nutritional interventions and growth factor therapies. Animal studies may......, newborn pigs and weanling rats represent a translational advantage for infant SBS due to their immature intestine. A balance among practical, economical, experimental and ethical constraints determines the choice of SBS model for each clinical or basic research question....

Hamartomatous polyposis syndromes

DEFF Research Database (Denmark)

Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

2014-01-01

Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes...

Contrast-Enhanced Ultrasound and Near-Infrared Spectroscopy of the Neonatal Bowel: Novel, Bedside, Noninvasive, and Radiation-Free Imaging for Early Detection of Necrotizing Enterocolitis.

Science.gov (United States)

Al-Hamad, Suzanne; Hackam, David J; Goldstein, Seth D; Huisman, Thierry A G M; Darge, Kassa; Hwang, Misun

2018-05-31

Despite extensive research and improvements in the field of neonatal care, the morbidity and mortality associated with necrotizing enterocolitis (NEC) have remained unchanged over the past three decades. Early detection of ischemia and necrotic bowel is vital in improving morbidity and mortality associated with NEC; however, strategies for predicting and preventing NEC are lacking. Contrast-enhanced ultrasound (CEUS) and near-infrared spectroscopy (NIRS) are novel techniques in pediatrics that have been proven as safe modalities. CEUS has benefits over conventional ultrasound (US) by its improved real-time evaluation of the micro- and macrovascularities of normally and abnormally perfused tissue. US has been implemented as a useful adjunct to X-ray for earlier evaluation of NEC. NIRS is another noninvasive technique that has shown promise in improving early detection of NEC. The purpose of this article is to review the current understanding of changes in bowel perfusion in NEC, discuss the accuracy of abdominal US in detecting NEC, and explain how the use of CEUS and NIRS will enhance the precise and early detection of altered/pathological bowel wall perfusion in the initial development and course of NEC. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Probiotics feeding in prevention of urinary tract infection, bacterial sepsis and necrotizing enterocolitis in preterm infants. A prospective double-blind study.

Science.gov (United States)

Dani, Carlo; Biadaioli, Roberto; Bertini, Giovanna; Martelli, Elena; Rubaltelli, Firmino F

2002-08-01

It has been suggested that probiotics can reduce the overgrowth of pathogens in the bowels of preterm infants and contribute to the reduction of the incidence of nosocomial infections in neonatal intensive care units (NICUs). The purpose of this study was to evaluate the effectiveness of Lactobacillus GG supplementation in reducing the incidence of urinary tract infections (UTIs), bacterial sepsis and necrotizing enterocolitis (NEC) in preterm infants. A double-blind study was conducted in 12 Italian NICUs. Newborn infants with a gestational age probiotics group (n = 295) and the placebo group (n = 290) exhibited similar clinical characteristics. The duration of Lactobacillus GG and placebo supplementation was 47.3 +/- 26.0 and 48.2 +/- 24.3 days, respectively. Although UTIs (3.4 vs. 5.8%) and NEC (1.4 vs. 2.7%) were found less frequently in the probiotic group compared to the control group, these differences were not significant. Bacterial sepsis was more frequent in the probiotics group (4.4%, n = 11) than in the placebo group (3.8%, n = 9), but the difference was not significant. Seven days of Lactobacillus GG supplementation starting with the first feed is not effective in reducing the incidence of UTIs, NEC and sepsis in preterm infants. Further studies are required to confirm our results in lower birthweight populations. Copyright 2002 S. Karger AG, Basel

Cost-effective optical fiber pressure sensor based on intrinsic Fabry-Perot interferometric micro-cavities

Science.gov (United States)

Domingues, M. Fátima; Rodriguez, Camilo A.; Martins, Joana; Tavares, Cátia; Marques, Carlos; Alberto, Nélia; André, Paulo; Antunes, Paulo

2018-05-01

In this work, a cost-effective procedure to manufacture optical fiber pressure sensors is presented. This has a high relevance for integration in robotic exoskeletons or for gait plantar pressure monitoring within the physical rehabilitation scenarios, among other applications. The sensing elements are based on Fabry-Perot interferometric (FPI) micro-cavities, created from the recycling of optical fibers previously destroyed by the catastrophic fuse effect. To produce the pressure sensors, the fiber containing the FPI micro-cavities was embedded in an epoxy resin cylinder used as pressure transducer and responsible to transfer the pressure applied on its surface to the optical fiber containing the FPI micro-cavity. Before the embedding process, some FPI sensors were also characterized to strain variations. After that, the effect of the encapsulation of the FPI structure into the resin was assessed, from which a slight decrease on the FPI interferogram fringes visibility was verified, indicating a small increase in the micro-cavity length. Up on the sensors characterization, a linear dependence of the wavelength shift with the induced pressure was obtained, which leads to a maximum sensitivity of 59.39 ± 1.7 pm/kPa. Moreover, direct dependence of the pressure sensitivity with the micro-cavity volume and length was found.

Effects of kinesiotaping on foot posture in participants with pronated foot: a quasi-randomised, double-blind study.

Science.gov (United States)

Luque-Suarez, Alejandro; Gijon-Nogueron, Gabriel; Baron-Lopez, Francisco Javier; Labajos-Manzanares, Maria Teresa; Hush, Julia; Hancock, Mark Jonathan

2014-03-01

To investigate whether kinesiotaping improves excessive foot pronation compared with sham kinesiotaping. Quasi-randomised, double-blind study. One primary care centre. One hundred and thirty participants were screened for inclusion. Sixty-eight participants with pronated feet [Foot Posture Index (FPI)≥ 6] were enrolled, and the follow-up rate was 100%. Participants were allocated into one of two groups: an experimental kinesiotaping group (KT1) and a sham taping group (KT2). Measures were collected by a blinded assessor at baseline, and 1 minute, 10 minutes, 60 minutes and 24 hours after taping. The primary outcome was total FPI score, and the secondary outcome was rear-foot FPI score. There were no significant differences in total FPI score between kinesiotaping and sham taping at any time point. Similarly, there were no significant differences in rear-foot FPI score, apart from at 60-minute follow-up when the difference between groups was significant (P=0.04) but the effect size was very small (0.85 points on the rear-foot FPI score between -6 and +6). Kinesiotaping does not correct foot pronation compared with sham kinesiotaping in people with pronated feet. Copyright © 2013 Chartered Society of Physiotherapy. All rights reserved.

Predicting glucose intolerance with normal fasting plasma glucose by the components of the metabolic syndrome

International Nuclear Information System (INIS)

Pei, D.; Lin, J.; Kuo, S.; Wu, D.; Li, J.; Hsieh, C.; Wu, C.; Hung, Y.; Kuo, K.

2007-01-01

Surprisingly it is estimated that about half of type 2 diabetics remain undetected. The possible causes may be partly attributable to people with normal fasting plasma glucose (FPG) but abnormal postprandial hyperglycemia. We attempted to develop an effective predictive model by using the metabolic syndrome (MeS) components as parameters to identify such persons. All participants received a standard 75 gm oral glucose tolerance test which showed that 106 had normal glucose tolerance, 61 had impaired glucose tolerance and 6 had diabetes on isolated postchallenge hyperglycemia. We tested five models which included various MeS components. Model 0: FPG; Model 1 (Clinical history model): family history (FH), FPG, age and sex; Model 2 (MeS model): Model 1 plus triglycerides, high-density lipoprotein cholesterol, body mass index, systolic blood pressure and diastolic blood pressure; Model 3: Model 2 plus fasting plasma insulin (FPI); Model 4: Model 3 plus homeostasis model assessment of insulin resistance. A receiver-operating characteristic (ROC) curve was used to determine the predictive discrimination of these models. The area under the ROC curve of the Model 0 was significantly larger than the area under the diagonal reference line. All the other 4 models had a larger area under the ROC curve than Model 0. Considering the simplicity and lower cost of Model 2, it would be the best model to use. Nevertheless, Model 3 had the largest area under the ROC curve. We demonstrated that Model 2 and 3 have a significantly better predictive discrimination to identify persons with normal FPG at high risk for glucose intolerance. (author)

Seckel syndrome: an overdiagnosed syndrome.

OpenAIRE

Thompson, E; Pembrey, M

1985-01-01

Five children in whom a diagnosis of Seckel syndrome had previously been made were re-examined in the genetic unit. One child had classical Seckel syndrome, a sib pair had the features of the syndrome with less severe short stature, and in two children the diagnosis was not confirmed. Seckel syndrome is only one of a group of low birth weight microcephalic dwarfism and careful attention should be paid to fulfillment of the major criteria defined by Seckel before the diagnosis is made. There r...

Efficacy of two regimens of dexamethasone for management of preterm labour: pilot study

International Nuclear Information System (INIS)

Rasool, A.; Farooq, U.

2017-01-01

Background: Dexamethasone is widely used for prevention of respiratory distress syndrome (RDS), necrotising enterocolitis (NEC) and intra-ventricular haemorrhage (IVH) in preterm babies; decreasing the neonatal mortality rate. There is no consensus on the dose of corticosteroid administered to the mother expected to have a preterm baby. This study is conducted to compare the effectiveness of two popular regimens of dexamethasone administration in decreasing incidence of RDS, necrotizing enterocolitis, IVH and neonatal mortality rate. Methods: Randomized control trial was conducted at Ayub Teaching Hospital, Abbottabad from 1st to 31st August, 2014. Sample size was set at 50. Block randomization was employed in the trial to allocate the patients into corresponding groups 'A' and 'B'. Group A was administered 6mg dexamethasone in 4 doses 12 hours apart and group B was administered 2 doses 12 hours apart. Results: Forty-eight patients participated in the study with 24 patients in each group. Mean age and period gestation of participants were 28.4 years±4.3 SD and 34 weeks±1.9 SD respectively. Four patients in group A gave birth to neonate with RDS compared to two cases in group B. Group B had higher incidence of necrotizing enterocolitis and neonatal mortalities. However, none of these differences observed were statistically significant. No case of IVH was reported in either of the groups. Conclusion: Both the popular regimens of dexamethasone administration are equally effective in decreasing the incidence of neonatal diseases. (author)

Necrotising enterocolitis in preterm infants: epidemiology and antibiotic consumption in the Polish neonatology network neonatal intensive care units in 2009.

Directory of Open Access Journals (Sweden)

Jadwiga Wójkowska-Mach

Full Text Available The aim of this study was to describe the epidemiology of necrotising enterocolitis (NEC, antibiotic consumption and the usefulness of microbiological tests in very low birth weight (VLBW Polish newborns.Prospective surveillance was performed in the year 2009 by local infection control teams. The study covered 910 infants hospitalized in six Polish neonatal intensive care units. Two kinds of indicators were used for the description of antibiotic usage: the duration of treatment (days of treatment, DOTs and the defined daily dose (DDD.NEC incidence was 8.7% and fatality rate was 19%. Chorioamnionitis, late gestational age and low birth weight were identified as risk factors for NEC. Catheterization, mechanical ventilation and other selected procedures were used considerably longer in newborns with NEC than in the remaining neonates. Total usage of antibiotics reached 2.9 DDDs or 1.437 days; the average use of drugs per case of NEC amounted to 0.47 DDD or 23.2 DOTs. The level of antibiotic usage was analysed with correlation to microbiological tests performed and it was non-significantly greater in the group of children with NEC in whom the tests were performed.A high risk of developing NEC is closely associated with VLBW and with inflammation of the amnion during labour. We observed no relationship between the consumption of antibiotics in neonates with NEC and positive results of microbiological testing indicating sepsis accompanying NEC or gut colonization with pathogens.

Effects of acute restraint-induced stress on glucocorticoid receptors and brain-derived neurotrophic factor after mild traumatic brain injury.

Science.gov (United States)

Griesbach, G S; Vincelli, J; Tio, D L; Hovda, D A

2012-05-17

We have previously reported that experimental mild traumatic brain injury results in increased sensitivity to stressful events during the first post-injury weeks, as determined by analyzing the hypothalamic-pituitary-adrenal (HPA) axis regulation following restraint-induced stress. This is the same time period when rehabilitative exercise has proven to be ineffective after a mild fluid-percussion injury (FPI). Here we evaluated effects of stress on neuroplasticity. Adult male rats underwent either an FPI or sham injury. Additional rats were only exposed to anesthesia. Rats were exposed to 30 min of restraint stress, followed by tail vein blood collection at post-injury days (PID) 1, 7, and 14. The response to dexamethasone (DEX) was also evaluated. Hippocampal tissue was collected 120 min after stress onset. Brain-derived neurotrophic factor (BDNF) along with glucocorticoid (GR) and mineralocorticoid (MR) receptors was determined by Western blot analysis. Results indicated injury-dependent changes in glucocorticoid and mineralocorticoid receptors that were influenced by the presence of dexamethasone. Control and FPI rats responded differentially to DEX in that GR increases after receiving the lower dose of DEX were longer lasting in the FPI group. A suppression of MR was found at PID 1 in vehicle-treated FPI and Sham groups. Decreases in the precursor form of BDNF were observed in different FPI groups at PIDs 7 and 14. These findings suggest that the increased sensitivity to stressful events during the first post-injury weeks, after a mild FPI, has an impact on hippocampal neuroplasticity. Copyright © 2012 IBRO. Published by Elsevier Ltd. All rights reserved.

Current Knowledge of Necrotizing Enterocolitis in Preterm Infants and the Impact of Different Types of Enteral Nutrition Products12

Science.gov (United States)

Dicken, Bryan; Hartling, Lisa

2017-01-01

Preterm infants are extremely vulnerable to a range of morbidities and mortality. Underdeveloped cardiac, respiratory, gastrointestinal, and immune systems in the preterm period increase the risk of necrotizing enterocolitis (NEC), a serious disease of the gut. NEC affects 5–12% of very–low birth-weight infants, leads to surgery in 20–40% of cases, and is fatal in 25–50% of cases. There are multiple factors that may contribute to NEC, but the exact cause is not yet fully understood. Severe cases can result in intestinal resection or death, and the health care costs average >$300,000/infant when surgical management is required. Different types of nutrition may affect the onset or progression of NEC. Several studies have indicated that bovine milk–based infant formulas lead to a higher incidence of NEC in preterm infants than does human milk (HM). However, it is not clear why HM is linked to a lower incidence of NEC or why some infants fed an exclusively HM diet still develop NEC. An area that has not been thoroughly explored is the use of semielemental or elemental formulas. These specialty formulas are easy to digest and absorb in the gut and may be an effective nutritional intervention for reducing the risk of NEC. This review summarizes what is known about the factors that contribute to the onset and progression of NEC, discusses its health care cost implications, and explores the impact that different formulas and HM have on this disease. PMID:28096129

Superior Mesenteric Artery Syndrome or Wilkie Syndrome

International Nuclear Information System (INIS)

Castano Llano, Rodrigo; Chams Anturi, Abraham; Arango Vargas, Paula

2009-01-01

We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

Beals Syndrome

Science.gov (United States)

... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

Fluorinated polyimides grafted with poly(ethylene glycol) side chains by the RAFT-mediated process and their membranes

International Nuclear Information System (INIS)

Chen Yiwang; Chen Lie; Nie Huarong; Kang, E.T.; Vora, R.H.

2005-01-01

Graft polymerization of poly(ethylene glycol) methyl ether methacrylate (PEGMA) from fluorinated polyimide (FPI) was carried out by the reversible addition-fragmentation chain transfer (RAFT)-mediated process. The peroxides generated by the ozone treatment on FPI facilitated the thermally-initiated graft copolymerization from FPI backbone. The 'living' character of the graft chain growing was ascertained in the subsequent chain extension of PEGMA. Nuclear magnetic resonance (NMR) and molecular weight measurements were used to characterize the chemical composition and structure of the copolymers. Microfiltration (MF) membranes were fabricated from the FPI-g-PEGMA comb copolymers by phase inversion in aqueous media. Surface composition analysis of the membranes scanned by X-ray photoelectron spectroscopy (XPS) revealed a substantial surface enrichment of the hydrophilic components. The pore size distribution of the resulting membranes was found to be much more uniform than that of the corresponding membranes cast from FPI-g-PEGMA prepared by the conventional radical polymerization process in the absence of the chain transfer agent. The morphology of the membranes was characterized by scanning electron microscopy (SEM)

Serotonin syndrome

Science.gov (United States)

Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

[Association Budd Chiari syndrome, antiphospholipid syndrome and Grave's disease].

Science.gov (United States)

Mouelhi, Leila; Chaieb, Mouna; Debbeche, Radhouane; Salem, Mohamed; Sfar, Imene; Trabelsi, Sinda; Gorgi, Yosr; Najjar, Taoufik

2009-02-01

Antiphospholipid syndrome is revealed by Budd Chiari syndrome in 5% of the cases. Antiphospholipid syndrome is characterized by venous or arterial thrombosis, foetal loss and positivity of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies and anti-beta2-glycoprotein I. Anticardiolipin antibodies was reported in auto-immune thyroid disorders, particularly in Grave's disease. Antiphospholipid syndrome associated to Grave's disease was reported in only three cases. To describe a case report of association of Grave's disease and antiphospholipid syndrome. We report the first case of Grave's disease associated with antiphospholipid syndrome, revealed by Budd Chiari syndrome. Our observation is particular by the fact that it is about a patient presenting a Grave's disease associated with antiphospholipid syndrome revealed by Budd Chiari syndrome. This triple association has never been reported in literature. Although association between antiphospholipid syndrome and Grave's disease was previously described, further studies evaluating the coexistence of these two affections in the same patient would be useful.

Duane Syndrome

Science.gov (United States)

... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

Fluorescent Penetrant INSPECTION—CLEANING Study Update

Science.gov (United States)

Eisenmann, D.; Brasche, L.

2009-03-01

Fluorescent penetrant inspection (FPI) is widely used in the aviation industry and other industries for surface-breaking crack detection. As with all inspection methods, adherence to the process parameters is critical to the successful detection of defects. There is variety of lubricants and surface coatings used in the aviation industry which must be removed prior to FPI. Before the FPI process begins, components are cleaned using a variety of cleaning methods which are selected based on the alloy and the soil types which must be removed. It is also important that the cleaning process not adversely affect the FPI process. From the first three phases of this project it has been found that a hot water rinse can aid in the detection process when using this nondestructive method.

Wells syndrome and its relationship to Churg-Strauss syndrome.

Science.gov (United States)

Ratzinger, Gudrun; Zankl, Julia; Zelger, Bernhard

2013-08-01

  Wells syndrome has been described as an inflammatory disorder based on typical clinical appearance combined with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Churg-Strauss syndrome, on the other hand, is primarily a diffuse, necrotizing vasculitis but is also typically displaying eosinophils and flame figures. Despite several parallels, the present understanding of these two diseases excludes any pathogenetic relationship.   We describe the clinical course and histopathological appearance of three patients who had initially been diagnosed with Wells syndrome that developed into Churg-Strauss syndrome during the course of their disease.   The clinical presentation of all three patients led to the diagnosis of Wells syndrome by independent specialists. Histopathology showed an eosinophilic infiltrate and flame figures next to features of leukocytoclastic vasculitis. Detailed examination revealed asthma bronchiale and additional symptoms indicating Churg-Strauss syndrome. The initial diagnosis of Wells syndrome had to be revised to Churg-Strauss syndrome.   We conclude that Wells syndrome could be the starting point of a pathogenetic process that might reach its maximum in Churg-Strauss syndrome. As a clinical consequence, patients with Wells syndrome should be evaluated and followed for Churg-Strauss syndrome. © 2013 The International Society of Dermatology.

Goldenhar Syndrome in Association with Duane Syndrome

Directory of Open Access Journals (Sweden)

U D Shrestha

2012-03-01

Full Text Available Goldenhar syndrome (GHS is also known as Oculo-Auriculo-Vertebral (OAV syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error. Keywords: Duane retraction syndrome; goldenhar syndrome, refractive error.

Development of a Gestational Age-Specific Case Definition for Neonatal Necrotizing Enterocolitis.

Science.gov (United States)

Battersby, Cheryl; Longford, Nick; Costeloe, Kate; Modi, Neena

2017-03-01

Necrotizing enterocolitis (NEC) is a major cause of neonatal morbidity and mortality. Preventive and therapeutic research, surveillance, and quality improvement initiatives are hindered by variations in case definitions. To develop a gestational age (GA)-specific case definition for NEC. We conducted a prospective 34-month population study using clinician-recorded findings from the UK National Neonatal Research Database between December 2011 and September 2014 across all 163 neonatal units in England. We split study data into model development and validation data sets and categorized GA into groups (group 1, less than 26 weeks' GA; group 2, 26 to less than 30 weeks' GA; group 3, 30 to less than 37 weeks' GA; group 4, 37 or more weeks' GA). We entered GA, birth weight z score, and clinical and abdominal radiography findings as candidate variables in a logistic regression model, performed model fitting 1000 times, averaged the predictions, and used estimates from the fitted model to develop an ordinal NEC score and cut points to develop a dichotomous case definition based on the highest area under the receiver operating characteristic curves [AUCs] and positive predictive values [PPVs]. Abdominal radiography performed to investigate clinical concerns. Ordinal NEC likelihood score, dichotomous case definition, and GA-specific probability plots. Of the 3866 infants, the mean (SD) birth weight was 2049.1 (1941.7) g and mean (SD) GA was 32 (5) weeks; 2032 of 3663 (55.5%) were male. The total included 2978 infants (77.0%) without NEC and 888 (23.0%) with NEC. Infants with NEC in group 1 were less likely to present with pneumatosis (31.1% vs 47.2%; P = .01), blood in stool (11.8% vs 29.6%; P definition were 2 or greater for infants in groups 1 and 2, 3 or greater for infants in group 3, and 4 or greater for infants in group 4. The ordinal NEC score and dichotomous case definition discriminated well between infants with (AUC, 87%) and without (AUC, 80%) NEC. The case

Fanconi syndrome

Science.gov (United States)

De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

Intestinal alkaline phosphatase administration in newborns decreases systemic inflammatory cytokine expression in a neonatal necrotizing enterocolitis rat model.

Science.gov (United States)

Rentea, Rebecca M; Liedel, Jennifer L; Fredrich, Katherine; Welak, Scott R; Pritchard, Kirkwood A; Oldham, Keith T; Simpson, Pippa M; Gourlay, David M

2012-10-01

Supplementation of intestinal alkaline phosphatase (IAP), an endogenous protein expressed in the intestines, decreases the severity of necrotizing enterocolitis (NEC)-associated intestinal injury and permeability. We hypothesized that IAP administration is protective in a dose-dependent manner of the inflammatory response in a neonatal rat model. Pre- and full-term newborn Sprague-Dawley rat pups were sacrificed on day of life 3. Control pups were vaginally delivered and dam fed. Preterm pups were delivered via cesarean section and exposed to intermittent hypoxia and formula feeds containing lipopolysaccharide (NEC) with and without IAP. Three different standardized doses were administered to a group of pups treated with 40, 4, and 0.4U/kg of bovine IAP (NEC+IAP40, IAP4, or IAP0.4U). Reverse transcription-real-time polymerase chain reaction (RT-PCR) for inducible nitric oxide synthase (iNOS) and tumor necrosis factor (TNF)-α on liver and lung tissues and serum cytokine analysis for interleukin (IL)-1β, IL-6, IL-10, and TNF-α were performed. Data were analyzed by Kruskal-Wallis and Mann-Whitney tests, expressed as mean±standard error of the mean and P≤0.05 considered significant. Levels of cytokines IL-1β, IL-6, and TNF-α increased significantly in NEC versus control, returning to control levels with increasing doses of supplemental enteral IAP. Hepatic and pulmonary TNF-α and iNOS messenger ribonucleic acid expressions increased in NEC, and the remaining elevated despite IAP supplementation. Proinflammatory cytokine expression is increased systemically with intestinal NEC injury. Administration of IAP significantly reduces systemic proinflammatory cytokine expression in a dose-dependent manner. Early supplemental enteral IAP may reduce NEC-related injury and be useful for reducing effects caused by a proinflammatory cascade. Copyright © 2012 Elsevier Inc. All rights reserved.

OCULO-CEREBRO-RENAL SYNDROME (LOWE'S SYNDROME)

Institute of Scientific and Technical Information of China (English)

无

1991-01-01

Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.

Determination of the pion charge form factor for Q2 = 0.60-1.60 (GeV/c)2

International Nuclear Information System (INIS)

Vardan Tadevosyan; Henk Blok; Garth Huber; David Abbott; Heinz Anklin; Christopher Armstrong; John Arrington; Ketevi Assamagan; Steven Avery; O. Baker; C. Bochna; Edward Brash; Herbert Breuer; Nicholas Chant; James Dunne; T. Eden; Rolf Ent; David Gaskell; Ronald Gilman; Kenneth Gustafsson; Wendy Hinton; Harold Jackson; Mark Jones; Cynthia Keppel; Pyunghun Kim; Wooyoung Kim; Andreas Klein; Douglas Koltenuk; Meihua Liang; George Lolos; Allison Lung; David Mack; David McKee; David Meekins; Joseph Mitchell; Hamlet Mkrtchyan; Robert Mueller; Gabriel Niculescu; Maria-Ioana Niculescu; David Pitz; David Potterveld; Liming Qin; Joerg Reinhold; Ilkyoung Shin; Stepan Stepanyan; Liguang Tang; Rob van der Meer; Kelley Vansyoc; D. Van Westrum; Jochen Volmer; William Vulcan; Stephen Wood; Chen Yan; Wenxia Zhao; Benedikt Zihlmann

2006-01-01

The data analysis for the reaction H(e,e(prime) pi + )n, which was used to determine values for the charged pion form factor Fpi for values of Q2 = 0.6-1.6 (gEv/C) 2 , has been repeated with careful inspection of all steps and special attention to systematic uncertainties. Also the method used to extract Fpi from the measured longitudinal cross section was critically reconsidered. Final values for the separated longitudinal and transverse cross sections and the extracted values of Fpi are presented

Neurofibromatosis-Noonan syndrome or LEOPARD Syndrome? A clinical dilemma.

Directory of Open Access Journals (Sweden)

Tullu M

2000-04-01

Full Text Available Neurofibromatosis (NF, Noonan syndrome (NS, and LEOPARD syndrome are all autosomal dominant conditions, each being a distinct clinical entity by itself. Rarely, one encounters cases with features of NF and NS and is termed as the ′Neurofibromatosis-Noonan syndrome′ (NF-NS. The authors report a clinical dilemma with major clinical features of the NF-NS syndrome and LEOPARD syndrome co-existing in the same patient. Also, features of Noonan syndrome and LEOPARD syndrome are compared with the case reported.

LEOPARD syndrome is not linked to the Marfan syndrome and the Watson syndrome loci

Energy Technology Data Exchange (ETDEWEB)

Rass-Rothchild, A.: Abeliovitch, D.; Kornstein, A. [Tel Aviv Univ. (Israel)]|[Hebrew Univ., Jerusalem (Israel)

1994-09-01

The acronym LEOPARD stands for a syndromic association of Lentigines, Eletrocardiographic changes, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth and sensorineural Deafness. Inheritance is autosomal dominant with high penetrance and variable expressivity. In 1990 Torok et al. reported on the association of LEOPARD and Marfan syndrome. In addition a clinical similarity (cardiac and cutaneous involvement) exists with the Watson syndrome (neurofibromatosis and pulmonic stenosis) which is linked to the marker D17S33 on chromosome 17. We studied possible linkage of LEOPARD syndrome to the Marfan syndrome locus on chromosome 15 (D15S1, MF13, and (TAAAA)n repeats) and to the NF-1 locus on chromosome 17 in a family with 9 cases of LEOPARD syndrome. Close linkage between LEOPARD syndrome and both the Marfan locus on chromosome 15 and the NF-1 locus on chromosome 17 was excluded (lod score <-2.0 through {theta} = 0.1).

A study of drying and cleaning methods used in preparation for fluorescent penetrant inspection - Part II

International Nuclear Information System (INIS)

Brasche, L.; Lopez, R.; Larson, B.

2003-01-01

Fluorescent penetrant inspection is the most widely used method for aerospace components such as critical rotating components of gas turbine engines. Successful use of FPI begins with a clean and dry part, followed by a carefully controlled and applied FPI process, and conscientious inspection by well trained personnel. A variety of cleaning methods are in use for cleaning of titanium and nickel parts with selection based on the soils or contamination to be removed. Cleaning methods may include chemical or mechanical methods with sixteen different types studied as part of this program. Several options also exist for use in drying parts prior to FPI. Samples were generated and exposed to a range of conditions to study the effect of both drying and cleaning methods on the flaw response of FPI. Low cycle fatigue (LCF) cracks were generated in approximately 40 nickel and 40 titanium samples for evaluation of the various cleaning methods. Baseline measurements were made for each of the samples using a photometer to measure sample brightness and a UVA videomicroscope to capture digital images of the FPI indications. Samples were exposed to various contaminants, cleaned and inspected. Brightness measurements and digital images were also taken to compare to the baseline data. A comparison of oven drying to flash dry in preparation for FPI has been completed and will be reported in Part I. Comparison of the effectiveness of various cleaning methods for the contaminants will be presented in Part II. The cleaning and drying studies were completed in cooperation with Delta Airlines using cleaning, drying and FPI processes typical of engine overhaul processes and equipment. The work was completed as part of the Engine Titanium Consortium and included investigators from Honeywell, General Electric, Pratt and Whitney, and Rolls Royce

A study of drying and cleaning methods used in preparation for fluorescent penetrant inspection - Part I

International Nuclear Information System (INIS)

Brasche, L.; Lopez, R.; Larson, B.

2003-01-01

Fluorescent penetrant inspection is the most widely used method for aerospace components such as critical rotating components of gas turbine engines. Successful use of FPI begins with a clean and dry part, followed by a carefully controlled and applied FPI process, and conscientious inspection by well trained personnel. A variety of cleaning methods are in use for cleaning of titanium and nickel parts with selection based on the soils or contamination to be removed. Cleaning methods may include chemical or mechanical methods with sixteen different types studied as part of this program. Several options also exist for use in drying parts prior to FPI. Samples were generated and exposed to a range of conditions to study the effect of both drying and cleaning methods on the flaw response of FPI. Low cycle fatigue (LCF) cracks were generated in approximately 40 nickel and 40 titanium samples for evaluation of the various cleaning methods. Baseline measurements were made for each of the samples using a photometer to measure sample brightness and a UVA videomicroscope to capture digital images of the FPI indications. Samples were exposed to various contaminants, cleaned and inspected. Brightness measurements and digital images were also taken to compare to the baseline data. A comparison of oven drying to flash dry in preparation for FPI has been completed and will be reported in Part I. Comparison of the effectiveness of various cleaning methods for the contaminants will be presented in Part II. The cleaning and drying studies were completed in cooperation with Delta Airlines using cleaning, drying and FPI processes typical of engine overhaul processes and equipment. The work was completed as part of the Engine Titanium Consortium and included investigators from Honeywell, General Electric, Pratt and Whitney, and Rolls Royce

A quality of life quandary: a framework for navigating parental refusal of treatment for co-morbidities in infants with underlying medical conditions.

Science.gov (United States)

Kunz, Sarah N; McAdams, Ryan M; Diekema, Douglas S; Opel, Douglas J

2015-01-01

Parental refusal of a recommended treatment is not an uncommon scenario in the neonatal intensive care unit. These refusals may be based upon the parents' perceptions of their child's projected quality of life. The inherent subjectivity of quality of life assessments, however, can exacerbate disagreement between parents and healthcare providers. We present a case of parental refusal of surgical intervention for necrotizing enterocolitis in an infant with Bartter syndrome and develop an ethical framework in which to consider the appropriateness of parental refusal based upon an infant's projected quality of life. Copyright 2015 The Journal of Clinical Ethics. All rights reserved.

Enterocolite necrosante: resposta imflamatória x corticoterapia pré-natal

OpenAIRE

Precioso, Alexander Roberto; Proença, Renata Suman Mascaretti

2002-01-01

Necrotizing enterocolitis is the most frequently occurring gastrointestinal disorder in premature neonates. Animal models of necrotizing enterocolitis and prenatal administration of cortisone have demonstrated that cortisone may accelerate maturation of the mucosal barrier, therefore reducing the incidence of this gastrointestinal disorder. The authors present a review of the literature of the most important risk factors associated with necrotizing enterocolitis, such as inflammatory gastroin...

Preliminary Results of Cleaning Process for Lubricant Contamination

Science.gov (United States)

Eisenmann, D.; Brasche, L.; Lopez, R.

2006-03-01

Fluorescent penetrant inspection (FPI) is widely used for aviation and other components for surface-breaking crack detection. As with all inspection methods, adherence to the process parameters is critical to the successful detection of defects. Prior to FPI, components are cleaned using a variety of cleaning methods which are selected based on the alloy and the soil types which must be removed. It is also important that the cleaning process not adversely affect the FPI process. There are a variety of lubricants and surface coatings used in the aviation industry which must be removed prior to FPI. To assess the effectiveness of typical cleaning processes on removal of these contaminants, a study was initiated at an airline overhaul facility. Initial results of the cleaning study for lubricant contamination in nickel, titanium and aluminum alloys will be presented.

Preliminary Results of Cleaning Process for Lubricant Contamination

International Nuclear Information System (INIS)

Eisenmann, D.; Brasche, L.; Lopez, R.

2006-01-01

Fluorescent penetrant inspection (FPI) is widely used for aviation and other components for surface-breaking crack detection. As with all inspection methods, adherence to the process parameters is critical to the successful detection of defects. Prior to FPI, components are cleaned using a variety of cleaning methods which are selected based on the alloy and the soil types which must be removed. It is also important that the cleaning process not adversely affect the FPI process. There are a variety of lubricants and surface coatings used in the aviation industry which must be removed prior to FPI. To assess the effectiveness of typical cleaning processes on removal of these contaminants, a study was initiated at an airline overhaul facility. Initial results of the cleaning study for lubricant contamination in nickel, titanium and aluminum alloys will be presented

Metabolic syndrome and polycystic ovary syndrome: an intriguing overlapping.

Science.gov (United States)

Caserta, Donatella; Adducchio, Gloria; Picchia, Simona; Ralli, Eleonora; Matteucci, Eleonora; Moscarini, Massimo

2014-06-01

Metabolic syndrome is an increasing pathology in adults and in children, due to a parallel rise of obesity. Sedentary lifestyle, food habits, cultural influences and also a genetic predisposition can cause dyslipidemia, hypertension, abdominal obesity and insulin resistance which are the two main features of metabolic syndrome. Polycystic ovary syndrome (PCOS) is a condition directly associated with obesity, insulin resistance (HOMA index) and metabolic syndrome, and it is very interesting for its relationship and overlap with the metabolic syndrome. The relationship between the two syndromes is mutual: PCOS women have a higher prevalence of metabolic syndrome and also women with metabolic syndrome commonly present the reproductive/endocrine trait of PCOS. Prevention and treatment of metabolic syndrome and PCOS are similar for various aspects. It is necessary to treat excess adiposity and insulin resistance, with the overall goals of preventing cardiovascular disease and type 2 diabetes and improving reproductive failure in young women with PCOS. First of all, lifestyle changes, then pharmacological therapy, bariatric surgery and laparoscopic ovarian surgery represent the pillars for PCOS treatment.

Barber-Say syndrome and Ablepharon-Macrostomia syndrome: An overview

NARCIS (Netherlands)

de Maria, Beatrice; Mazzanti, Laura; Roche, Nathalie; Hennekam, Raoul C.

2016-01-01

Barber-Say syndrome (BSS) and Ablepharon-Macrostomia syndrome (AMS) are congenital malformation syndromes caused by heterozygous mutations in TWIST2. Here we provide a critical review of all patients published with these syndromes. We excluded several earlier reports due to misdiagnosis or

Autosomal dominant syndrome resembling Coffin-Siris syndrome.

Science.gov (United States)

Flynn, Maureen A; Milunsky, Jeff M

2006-06-15

Coffin-Siris syndrome is a multiple congenital anomaly/mental retardation syndrome with phenotypic variability [OMIM 135900]. The diagnosis is based solely on clinical findings, as there is currently no molecular, biochemical, or cytogenetic analysis available to confirm a diagnosis. Although typically described as an autosomal recessive disorder, autosomal dominant inheritance has also been infrequently reported. We describe a mother and her two daughters who all have features that resemble Coffin-Siris syndrome. However, this is not a completely convincing diagnosis given that hypertelorism is not a feature of Coffin-Siris syndrome and the family is relatively mildly affected. Yet, this family provides further evidence of an autosomal dominant mode of inheritance for a likely variant of Coffin-Siris syndrome (at least in some families). In addition, Sibling 1 had premature thelarche. She is the second reported individual within the spectrum of Coffin-Siris syndrome to have premature thelarche, indicating that it may be a rare clinical feature. Copyright 2006 Wiley-Liss, Inc.

West syndrome in a patient with Schinzel-Giedion syndrome.

Science.gov (United States)

Miyake, Fuyu; Kuroda, Yukiko; Naruto, Takuya; Ohashi, Ikuko; Takano, Kyoko; Kurosawa, Kenji

2015-06-01

Schinzel-Giedion syndrome is a rare recognizable malformation syndrome defined by characteristic facial features, profound developmental delay, severe growth failure, and multiple congenital anomalies. The causative gene of Schinzel-Giedion syndrome, SETBP1, has been identified, but limited cases have been confirmed by molecular analysis. We present a 9-month-old girl affected by West syndrome with Schinzel-Giedion syndrome. Congenital severe hydronephrosis, typical facial features, and multiple anomalies suggested a clinical diagnosis of Schinzel-Giedion syndrome. Hypsarrhythmia occurred at 7 months of age and was temporarily controlled by adrenocorticotropic hormone (ACTH) therapy during 5 weeks. SETBP1 mutational analysis showed the presence of a recurrent mutation, p.Ile871Thr. The implications in management of Schinzel-Giedion syndrome are discussed. © The Author(s) 2014.

Cow's milk allergy in children

African Journals Online (AJOL)

is primarily caused by dryness of the skin and is linked to hereditary factors. However ... in Table I. FPI disorders are typically cow's milk and soya protein induced3 but may also ... fish, chicken, turkey, corn and vegetables. FPI enteropathy ...

Cushing syndrome

Science.gov (United States)

Hypercortisolism; Cortisol excess; Glucocorticoid excess - Cushing syndrome ... The most common cause of Cushing syndrome is taking too much ... Cushing syndrome . Prednisone, dexamethasone, and prednisolone ...

Russell-Silver syndrome

Science.gov (United States)

Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome ... One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11. Most of the time, it ...

[Cockett's syndrome, May-Thurner syndrome, or iliac vein compression syndrome].

Science.gov (United States)

Gil Martín, A R; Carreras Aja, M; Arrieta Ardieta, I; Labayen Azparren, I

2014-01-01

Iliac vein compression syndrome (also known as May-Thurner syndrome or Cockett's syndrome) is a rare clinical entity in which the left common iliac vein is compressed when it passes between the right common iliac artery and the spine. The sustained compression and trauma caused by the pulsatile force of the artery on the vein damage the intima and lead to the formation of membranes or bands in the vascular lumen that hinder or obstruct the flow of blood in the vein, favoring thrombus formation. The current treatment strategy of choice is endovascular vein patch angioplasty and stenting with the aim of improving the caliber of the lumen and enabling normal venous drainage. We present two cases of May-Thurner syndrome and review the clinical and CT findings. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

LEOPARD syndrome

Science.gov (United States)

Multiple lentigines syndrome; Noonan syndrome with multiple lentigines ... Genetics Home Reference -- ghr.nlm.nih.gov/condition/noonan-syndrome-with-multiple-lentigines National Organization for Rare Disorders -- ...

Dravets syndrom

DEFF Research Database (Denmark)

Hansen, Lars Kjaersgård; Rasmussen, Niels Henrik; Ousager, Lilian Bomme

2010-01-01

Dravet syndrome is an epileptic syndrome of infancy and early childhood. Most cases of Dravet syndrome seem to be due to a genetic defect causing the sodium channel to malfunction. We describe the main features of the syndrome. This epilepsy is medically intractable, but we call attention...... to the fact that some medications are of benefit and some could exacerbate the condition. Early recognition of the syndrome including by genetic testing could possibly improve outcome and reduce the need for other specialized investigations. Udgivelsesdato: 2010-Feb-22...

A Chinese patient with pusher syndrome and unilateral spatial neglect syndrome.

Science.gov (United States)

Chen, Xiao-Wei; Lin, Cheng-He; Zheng, Hua; Lin, Zhen-Lan

2014-07-01

To observe clinical manifestations, behavioral characteristics, and effects of rehabilitation on a patient with pusher syndrome and unilateral spatial neglect caused by right thalamic hemorrhage. Assessment of pusher syndrome was made by the Scale for Contraversive pushing (SCP), and unilateral spatial neglect syndrome was diagnosed using line cancellation, letter and star cancellation, line bisection tests and copy and continuation of graphic sequence test. Behavioral therapy, occupational therapy, reading training and traditional Chinese medicine methods were adopted for treatment of pusher syndrome and unilateral spatial neglect. The patient showed typical pusher syndrome and unilateral spatial neglect symptoms. The pusher syndrome and unilateral spatial neglect symptoms were significantly improved following rehabilitation treatments. Pusher syndrome and unilateral spatial neglect syndrome occurred simultaneously after right thalamic hemorrhage. Early rehabilitation therapy can reduce the symptoms of pusher syndrome and unilateral spatial neglect syndrome and improve motor function.

Aarskog syndrome

Science.gov (United States)

Aarskog disease; Aarskog-Scott syndrome; AAS; Faciodigitogenital syndrome; Gaciogenital dysplasia ... Aarskog syndrome is a genetic disorder that is linked to the X chromosome. It affects mainly males, but females ...

Tracheostomy Among Infants With Hypoplastic Left Heart Syndrome Undergoing Cardiac Operations: A Multicenter Analysis.

Science.gov (United States)

Prodhan, Parthak; Agarwal, Amit; ElHassan, Nahed O; Bolin, Elijah H; Beam, Brandon; Garcia, Xiomara; Gaies, Michael; Tang, Xinyu

2017-04-01

Less than 2.7% of infants undergoing congenital heart disease operations have difficulty weaning from invasive mechanical ventilation. In such instances, clinicians may choose to perform tracheostomy. Limited literature has examined tracheostomy placement specifically in infants with hypoplastic left heart syndrome (HLHS). This study evaluated the risk factors for tracheostomy placement in infants with HLHS and examined the outcomes of these infants before their first hospital discharge. This retrospective analysis of the Pediatric Heath Information System data set included infants with HLHS who underwent stage 1 Norwood operation, a hybrid procedure, or heart transplant from 2004 through 2013. We identified 5721 infants with HLHS, and 126 underwent tracheostomy placement. Infants in the tracheostomy group had more morbidities and a higher mortality rate across the study period. Diagnosis of chromosomal abnormalities, anomalies of the trachea and esophagus, larynx, diaphragm and nervous system, bilateral vocal cord paralysis, and necrotizing enterocolitis, and procedures including extracorporeal membrane oxygenation support, cardiac catheterization, and gastrostomy tube were independently associated with tracheostomy placement in the study population. Despite an overall increase in rates of tracheostomy performed in infants with HLHS during the study period, the mortality rate did not improve among tracheostomy patients. Several risk factors were identified in infants with HLHS in whom a tracheostomy was placed during their first hospitalization. Despite an overall increase in rates of tracheostomies during the study period, the mortality rate did not improve among these patients. Appropriate family counseling and thorough preoperative case selection is suggested when discussing possible tracheostomy placement in infants with HLHS. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

Science.gov (United States)

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

OpenAIRE

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Cushing's Syndrome

OpenAIRE

宗, 友厚; 伊藤, 勇; 諏訪, 哲也; 武田, 純; MUNE, Tomoatsu

2003-01-01

Sixteen cases of verified Cushing's syndrome, and twelve cases of probable Cushing's syndrome were reviewed and data on them were compared with various reports on Cushing's syndrome in the literature.

A feature point identification method for positron emission particle tracking with multiple tracers

Energy Technology Data Exchange (ETDEWEB)

Wiggins, Cody, E-mail: cwiggin2@vols.utk.edu [University of Tennessee-Knoxville, Department of Physics and Astronomy, 1408 Circle Drive, Knoxville, TN 37996 (United States); Santos, Roque [University of Tennessee-Knoxville, Department of Nuclear Engineering (United States); Escuela Politécnica Nacional, Departamento de Ciencias Nucleares (Ecuador); Ruggles, Arthur [University of Tennessee-Knoxville, Department of Nuclear Engineering (United States)

2017-01-21

A novel detection algorithm for Positron Emission Particle Tracking (PEPT) with multiple tracers based on optical feature point identification (FPI) methods is presented. This new method, the FPI method, is compared to a previous multiple PEPT method via analyses of experimental and simulated data. The FPI method outperforms the older method in cases of large particle numbers and fine time resolution. Simulated data show the FPI method to be capable of identifying 100 particles at 0.5 mm average spatial error. Detection error is seen to vary with the inverse square root of the number of lines of response (LORs) used for detection and increases as particle separation decreases. - Highlights: • A new approach to positron emission particle tracking is presented. • Using optical feature point identification analogs, multiple particle tracking is achieved. • Method is compared to previous multiple particle method. • Accuracy and applicability of method is explored.

Polycystic ovary syndrome and metabolic syndrome.

Science.gov (United States)

Ali, Aus Tariq

2015-08-01

Polycystic ovary syndrome (PCOS) is a heterogeneous disorder, where the main clinical features include menstrual irregularities, sub-fertility, hyperandrogenism, and hirsutism. The prevalence of PCOS depends on ethnicity, environmental and genetic factors, as well as the criteria used to define it. On the other hand, metabolic syndrome is a constellation of metabolic disorders which include mainly abdominal obesity, insulin resistance, impaired glucose metabolism, hypertension and dyslipidaemia. These associated disorders directly increase the risk of Type 2 diabetes mellitus (DMT2), coronary heart disease (CHD), cardiovascular diseases (CVD) and endometrial cancer. Many patients with PCOS have features of metabolic syndrome such as visceral obesity, hyperinsulinaemia and insulin resistance. These place patients with PCOS under high risk of developing cardiovascular disease (CVD), Type 2 diabetes (DMT2) and gynecological cancer, in particular, endometrial cancer. Metabolic syndrome is also increased in infertile women with PCOS. The aim of this review is to provide clear and up to date information about PCOS and its relationship with metabolic syndrome, and the possible interaction between different metabolic disorders.

Tourette syndrome

Science.gov (United States)

Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. ...

Metabolic syndrome in acute coronary syndrome

International Nuclear Information System (INIS)

Bhalli, M.A.; Aamir, M.; Mustafa, G.

2011-01-01

Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

Metabolic syndrome in acute coronary syndrome

Energy Technology Data Exchange (ETDEWEB)

Bhalli, M A; Aamir, M; Mustafa, G [Combined Military Hospital, Abbottabad (Pakistan)

2011-06-15

Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

Exogenous Cushing syndrome

Science.gov (United States)

Cushing syndrome - corticosteroid induced; Corticosteroid-induced Cushing syndrome; Iatrogenic Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the hormone ...

Targets to treat metabolic syndrome in polycystic ovary syndrome.

Science.gov (United States)

Mahalingaiah, Shruthi; Diamanti-Kandarakis, Evanthia

2015-01-01

Metabolic syndrome is comprised of a combination of the following states: increased insulin resistance, dyslipidemia, cardiovascular disease, and increased abdominal obesity. Women with polycystic ovary syndrome (PCOS) have an increased risk of developing metabolic syndrome over the course of their lives. Metabolic syndrome increases risk of major cardiovascular events, morbidity, quality of life, and overall health care costs. Though metabolic syndrome in women with PCOS is an area of great concern, there is no effective individual medical therapeutic to adequately treat this issue. This article will review key aspects of metabolic syndrome in PCOS. We will discuss classic and novel therapeutics to address metabolic syndrome in women with PCOS. We will conclude with the importance of developing strategic interventions to increase the compliance to lifestyle and dietary modification, in addition to appreciation of the emerging pharmaceutical therapeutics available. Innovation in lifestyle modification, including diet, exercise, with and without dedicated stress reduction techniques is the future in treatment of metabolic syndrome in PCOS. Application of novel interventions, such as group medical care, may improve future adherence to lifestyle modification recommendations, in addition to or in combination with pharmaceutical therapeutics.

Targets to treat metabolic syndrome in polycystic ovary syndrome

Science.gov (United States)

Mahalingaiah, Shruthi; Diamanti-Kandarakis, Evanthia

2016-01-01

Introduction Metabolic syndrome is comprised of a combination of the following states: increased insulin resistance, dyslipidemia, cardiovascular disease, and increased abdominal obesity. Women with polycystic ovary syndrome (PCOS) have an increased risk of developing metabolic syndrome over the course of their lives. Metabolic syndrome increases risk of major cardiovascular events, morbidity, quality of life, and overall health care costs. Though metabolic syndrome in women with PCOS is an area of great concern, there is no effective individual medical therapeutic to adequately treat this issue. Areas Covered This article will review key aspects of metabolic syndrome in PCOS. We will discuss classic and novel therapeutics to address metabolic syndrome in women with PCOS. We will conclude with the importance of developing strategic interventions to increase the compliance to lifestyle and dietary modification, in addition to appreciation of the emerging pharmaceutical therapeutics available. Expert Opinion Innovation in lifestyle modification, including diet, exercise, with and without dedicated stress reduction techniques is the future in treatment of metabolic syndrome in PCOS. Application of novel interventions, such as group medical care, may improve future adherence to lifestyle modification recommendations, in addition to or in combination with pharmaceutical therapeutics. PMID:26488852

Concurrent Van der Woude syndrome and Turner syndrome: A case report.

Science.gov (United States)

Los, Evan; Baines, Hayley; Guttmann-Bauman, Ines

2017-01-01

Most cases of Van der Woude syndrome are caused by a mutation to interferon regulatory factor 6 on chromosome 1. Turner syndrome is caused by complete or partial absence of the second sex chromosome in girls. We describe a unique case of the two syndromes occurring concurrently though apparently independently in a girl with Van der Woude syndrome diagnosed at birth and Turner syndrome at 14 years 9 months. Short stature was initially misattributed to Van der Woude syndrome and pituitary insufficiency associated with clefts before correctly diagnosing Turner syndrome. We discuss the prevalence of delayed diagnosis of Turner syndrome, the rarity of reports of concurrent autosomal chromosome mutation and sex chromosome deletion, as well as the need to consider the diagnosis of Turner syndrome in all girls with short stature regardless of prior medical history.

Milk-alkali syndrome

Science.gov (United States)

Calcium-alkali syndrome; Cope syndrome; Burnett syndrome; Hypercalcemia; Calcium metabolism disorder ... Milk-alkali syndrome is almost always caused by taking too many calcium supplements, usually in the form of calcium carbonate. Calcium ...

Marfan Syndrome

Science.gov (United States)

Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

Brief Report: Repetitive Behaviour Profiles in Williams Syndrome: Cross Syndrome Comparisons with Prader-Willi and Down Syndromes

Science.gov (United States)

Royston, R.; Oliver, C.; Moss, J.; Adams, D.; Berg, K.; Burbidge, C.; Howlin, P.; Nelson, L.; Stinton, C.; Waite, J.

2018-01-01

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were…

Loeys-Dietz Syndrome

Science.gov (United States)

... to the signs and symptoms of Loeys-Dietz syndrome. Marfan syndrome is different from Loeys-Dietz syndrome in that the gene mutation which causes Marfan syndrome is in fibrillin-1 (FBN-1), a protein ...

Pre-Menstrual Syndrome in Women with Down Syndrome

Science.gov (United States)

Mason, Linda; Cunningham, Cliff

2009-01-01

Background: Prevalence of pre-menstrual syndrome (PMS) may be higher in women with Down syndrome due to syndrome specific characteristics in biochemistry, psychopathology and lifestyle. Recognition of PMS may be difficult for women with intellectual disabilities and their carers. Method: A daily diary, used to diagnose PMS with typical women, was…

Williams syndrome

Science.gov (United States)

Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

Coexistence of Reverse Capgras Syndrome, Subjective Double and Cotard Syndrome

Directory of Open Access Journals (Sweden)

Azadeh Mashayekhi

2016-01-01

Full Text Available Misidentification syndrome is a condition in which the person thinks that familiar persons have been replaced with other one. Coexistence of some types of this syndrome has been reported with other psychiatric syndromes. In this report, we present a 47-year-old married man with coexistence of reverse Capgras and subjective double syndromes with Cotard syndrome. There is no previous report of coexistence of these three forms of delusions in a single case.

28 CFR 345.20 - Position classification.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Position classification. 345.20 Section... INDUSTRIES (FPI) INMATE WORK PROGRAMS Position Classification § 345.20 Position classification. (a) Inmate... the objectives and principles of pay classification as a part of the routine orientation of new FPI...

Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome).

Science.gov (United States)

Bresler, Scott C; Padwa, Bonnie L; Granter, Scott R

2016-06-01

Nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome (Gorlin syndrome), is a rare autosomal dominantly inherited disorder that is characterized by development of basal cell carcinomas from a young age. Other distinguishing clinical features are seen in a majority of patients, and include keratocystic odontogenic tumors (formerly odontogenic keratocysts) as well as dyskeratotic palmar and plantar pitting. A range of skeletal and other developmental abnormalities are also often seen. The disorder is caused by defects in hedgehog signaling which result in constitutive pathway activity and tumor cell proliferation. As sporadic basal cell carcinomas also commonly harbor hedgehog pathway aberrations, therapeutic agents targeting key signaling constituents have been developed and tested against advanced sporadically occurring tumors or syndromic disease, leading in 2013 to FDA approval of the first hedgehog pathway-targeted small molecule, vismodegib. The elucidation of the molecular pathogenesis of nevoid basal cell carcinoma syndrome has resulted in further understanding of the most common human malignancy.

Aicardi Syndrome

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... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) × Definition Aicardi syndrome is a rare genetic ... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) View Full Definition Treatment There is no ...

Classification of Porcine Cranial Fracture Patterns Using a Fracture Printing Interface,.

Science.gov (United States)

Wei, Feng; Bucak, Serhat Selçuk; Vollner, Jennifer M; Fenton, Todd W; Jain, Anil K; Haut, Roger C

2017-01-01

Distinguishing between accidental and abusive head trauma in children can be difficult, as there is a lack of baseline data for pediatric cranial fracture patterns. A porcine head model has recently been developed and utilized in a series of studies to investigate the effects of impact energy level, surface type, and constraint condition on cranial fracture patterns. In the current study, an automated pattern recognition method, or a fracture printing interface (FPI), was developed to classify cranial fracture patterns that were associated with different impact scenarios documented in previous experiments. The FPI accurately predicted the energy level when the impact surface type was rigid. Additionally, the FPI was exceedingly successful in determining fractures caused by skulls being dropped with a high-level energy (97% accuracy). The FPI, currently developed on the porcine data, may in the future be transformed to the task of cranial fracture pattern classification for human infant skulls. © 2016 American Academy of Forensic Sciences.

Fournier gangrene associated with hyper IgE syndrome (Job syndrome).

Science.gov (United States)

Hori, Junichi; Yamaguchi, Satoshi; Watanabe, Masaki; Osanai, Hiroaki; Hori, Masako

2008-04-01

We report a case of a 32-year-old man with hyper IgE syndrome (Job syndrome) who developed Fournier gangrene due to infectious multiple atheromas of the scrotal skin that progressed to the right groin and thigh. The patient required surgical debridement and subsequent skin grafting. This is a rare case of Fournier gangrene associated with hyper IgE syndrome (Job syndrome). When a patient without diabetes mellitus has repeated infections and atopic-like dermatitis, Job syndrome should be considered.

Down Syndrome

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... Down syndrome increases as a woman gets older. Down syndrome cannot be cured. Early treatment programs can help improve skills. They may include ... occupational, and/or educational therapy. With support and treatment, many ... Down syndrome live happy, productive lives. NIH: National Institute of ...

TAFRO Syndrome.

Science.gov (United States)

Igawa, Takuro; Sato, Yasuharu

2018-02-01

TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

Development and characteristics of children with Usher syndrome and CHARGE syndrome.

Science.gov (United States)

Dammeyer, Jesper

2012-09-01

Individuals with Usher syndrome or CHARGE syndrome are faced with a number of difficulties concerning hearing, vision, balance, and language development. The aim of the study is to describe the developmental characteristics of children with Usher syndrome and CHARGE syndrome, respectively. Data about the developmental characteristics of 26 children with Usher syndrome and 17 children with CHARGE syndrome was obtained. Associations between deafblindness (dual sensory loss), motor development (age of walking), language abilities, and intellectual outcome of these children were explored for each group independently. Both groups of children face a number of difficulties associated with vision, hearing, language, balance and intellectual outcome. Intellectual disability and/or language delay was found among 42% of the children with Usher syndrome and among 82% of the children with CHARGE syndrome. Intellectual disability was associated with language delay and age of walking for both groups. Even though Usher and CHARGE are two different genetic syndromes, both groups are challenged with a number of similar developmental delays. Clinicians need to be aware of several developmental issues in order to offer adequate support to children with Usher or CHARGE syndrome. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Pregnancy outcome in joint hypermobility syndrome and Ehlers-Danlos syndrome.

Science.gov (United States)

Sundelin, Heléne E K; Stephansson, Olof; Johansson, Kari; Ludvigsson, Jonas F

2017-01-01

An increased risk of preterm birth in women with joint hypermobility syndrome or Ehlers-Danlos syndrome is suspected. In this nationwide cohort study from 1997 through 2011, women with either joint hypermobility syndrome or Ehlers-Danlos syndrome or both disorders were identified through the Swedish Patient Register, and linked to the Medical Birth Register. Thereby, 314 singleton births to women with joint hypermobility syndrome/Ehlers-Danlos syndrome before delivery were identified. These births were compared with 1 247 864 singleton births to women without a diagnosis of joint hypermobility syndrome/Ehlers-Danlos syndrome. We used logistic regression, adjusted for maternal age, smoking, parity, and year of birth, to calculate adjusted odds ratios for adverse pregnancy outcomes. Maternal joint hypermobility syndrome/Ehlers-Danlos syndrome was not associated with any of our outcomes: preterm birth (adjusted odds ratio = 0.6, 95% confidence interval 0.3-1.2), preterm premature rupture of membranes (adjusted odds ratio = 0.8; 95% confidence interval 0.3-2.2), cesarean section (adjusted odds ratio = 0.9, 95% confidence interval 0.7-1.2), stillbirth (adjusted odds ratio = 1.1, 95% confidence interval 0.2-7.9), low Apgar score (adjusted odds ratio = 1.6, 95% confidence interval 0.7-3.6), small for gestational age (adjusted odds ratio = 0.9, 95% confidence interval 0.4-1.8) or large for gestational age (adjusted odds ratio = 1.2, 95% confidence interval 0.6-2.1). Examining only women with Ehlers-Danlos syndrome (n = 62), we found a higher risk of induction of labor (adjusted odds ratio = 2.6; 95% confidence interval 1.4-4.6) and amniotomy (adjusted odds ratio = 3.8; 95% confidence interval 2.0-7.1). No excess risks for adverse pregnancy outcome were seen in joint hypermobility syndrome. Women with joint hypermobility syndrome/Ehlers-Danlos syndrome do not seem to be at increased risk of adverse pregnancy outcome. © 2016 Nordic Federation of

Momentum and hamiltonian in complex action theory

DEFF Research Database (Denmark)

Nagao, Keiichi; Nielsen, Holger Frits Bech

2012-01-01

$-parametrized wave function, which is a solution to an eigenvalue problem of a momentum operator $\\hat{p}$, in FPI with a starting Lagrangian. Solving the eigenvalue problem, we derive the momentum and Hamiltonian. Oppositely, starting from the Hamiltonian we derive the Lagrangian in FPI, and we are led...

Wolf-Hirschhorn (4p-) syndrome with West syndrome.

Science.gov (United States)

Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

2016-01-01

Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

Rowell syndrome

Directory of Open Access Journals (Sweden)

Ramesh Y Bhat

2014-01-01

Full Text Available Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine.

[Poland's syndrome].

Science.gov (United States)

Slezak, R; Sasiadek, M

2000-08-01

Poland's syndrome consists of the variable clinical features, but always includes unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. The incidence of Poland's syndrome, reported by different authors ranges from 1:10,000 to 1:100,000 and is observed more frequently in males than in females with the right side of the body affected more often than the left. The etiology of this syndrome is still discussed. However most of described cases were sporadic, rare familial incidence of Poland's syndrome were also presented. Therefore different etiologic factors of the Poland's syndrome are taken into account: genetic, vascular compromise during early stages of embriogenesis but also teratogenic effect of environmental xenobiotics (e.g. cigarette smoking by pregnant women). The authors present also the case of 20-years old man with inherited bilateral syndactyly with the right side aplasia of major pectoralis muscle and face asymmetry. The familial history was negative in respect to the features, associated with Poland's syndrome.

[Gardner syndrome--parent alienation syndrome (PAS). Diagnosis or family reality?].

Science.gov (United States)

Namysłowska, Irena; Heitzman, Janusz; Siewierska, Anna

2009-01-01

The authors present characteristics of Parental Alienation Syndrome (PAS) proposed by Gardner as well as data, which may help to differentiate that syndrome with real psychological, physical and sexual abuse. The consequences of Gardner Syndrome for legal decisions in the court cases of child custody and the critique of this syndrome in forensic and psychiatric literature are also discussed, and several questions posed. Authors propose to treat Gardner Syndrome not as as a child disorder but as a specific, dynamic family situation, which occurs sometimes, during divorce and fight about child custody.

Burning Mouth Syndrome and "Burning Mouth Syndrome".

Science.gov (United States)

Rifkind, Jacob Bernard

2016-03-01

Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome.

Marfan Syndrome (For Teens)

Science.gov (United States)

... genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, the French ... immediately. What's Life Like for Teens With Marfan Syndrome? Marfan syndrome affects people differently, so life is not ...

Learning about Marfan Syndrome

Science.gov (United States)

... Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most common inherited ... FAQ Top of page Additional Resources For Marfan Syndrome Marfan syndrome [nlm.nih.gov] From Medline Plus Marfan ...

Divorce in families of children with Down Syndrome or Rett Syndrome.

Science.gov (United States)

Lederman, Vivian Renne Gerber; Alves, Bianca dos Santos; Negrão, Juliana; Maria, Juliana Negrão; Schwartzman, José Salomão; D'Antino, Maria Eloisa Famá; Brunoni, Decio

2015-05-01

This study evaluates the impact in the stability and management of the marriage of parents of a child with Down or Rett Syndrome. Morbidity of the syndromes and the marital status of the couples before and after the birth of the affected children were considered variables. The divorce rate in families with Down syndrome was 10%, similar to the Brazilian rate population. In Rett Syndrome, the divorce rate was significantly higher, 23.5%. The higher morbidity of Rett Syndrome, and the moment of diagnosis could be relevant factors for the increased divorce rate related to this syndrome.

The sick-building syndrome; Das Sick-Building-Syndrom

Energy Technology Data Exchange (ETDEWEB)

Henne, A.; Neumann, H.F.; Winneke, G.

1992-12-31

The sick-building syndrome is characterized by the presence of general, non-specific symptoms (e.g., headache, tiredness, respiratory problems, eye trouble, vertigo, nausea, unspecific hypersensitivity) in association with a particular indoor ambience. It is clearly distinguishable from `building-related illness`, referring to a well-defined clinical syndrome due to staying in a building and for which a cause can, in general, be established. Disorders in the case of the sick-building syndrome are manifold and confirmed objectifiable results are hardly available so far. Yet there are some organ-related methods for the confirmation of findings concerning, for instance, the eyes, the skin and the area of the nose. The causes of the incidence of sick-building syndrome are more or less unclear. It is a multifactorial phenomenon involving physical, biological, chemical, individual-specific and psychological factors. Buildings where sick-building syndrome occurs typically exhibit certain properties. The European Community has already made proposals for the investigation of incriminated buildings. A systematic survey by questionnaire together with individual interviews plays an import part towards clarifying the syndrome. (orig./UWA) [Deutsch] Das Sick-Building-Syndrom beschreibt das Vorhandensein von allgemeinen, nicht spezifischen Symptomen (z.B. Kopfschmerzen, Muedigkeit, Atembeschwerden, Augenreizungen, Schwindelgefuehl, Uebelkeit, unspezifische Ueberempfindlichkeit), assoziiert mit einer besonderen Innenraumumgebung. Deutlich hiervon abzugrenzen ist die ``Building related illness``, bei der ein klinisch definiertes Krankheitsbild vorliegt, das durch den Aufenthalt im Gebaeude verursacht wird und fuer das im allgemeinen eine Ursache ermittelt werden kann. Das Beschwerdebild beim Sick-Building-Syndrom ist vielfaeltig, und gesicherte, objektivierbare Befunde liegen hierzu bisher kaum vor. Dennoch gibt es einige organbezogenen Methoden zur Befundabsicherung, z.B. fuer das

Hypokalaemia: Bartter's syndrome or pseudo-Bartter's syndrome?

OpenAIRE

Robb, J D; Delargy, M A; Nolan, M; Tomkin, G H

1984-01-01

The difficulties in the diagnosis of hypokalaemia are often considerable. This paper reports three patients who presented with hypokalaemia. Investigations are described which may help to distinguish Bartter's syndrome from pseudo-Bartter's syndrome.

Delayed initiation but not gradual advancement of enteral formula feeding reduces the incidence of necrotizing enterocolitis (NEC in preterm pigs.

Directory of Open Access Journals (Sweden)

Nada Ghoneim

Full Text Available Enteral formula feeding is a risk factor for necrotizing enterocolitis (NEC in premature infants, yet studies are conflicting regarding the safest timing for introduction and advancement of feeds. Our aim was to test the effects of early vs. late initiation and abrupt vs. gradual advancement of enteral feeding of an intact vs. hydrolyzed protein formula on NEC incidence and severity in preterm pigs. In Experiment 1, preterm pigs received total parenteral nutrition (TPN at birth with abrupt initiation of enteral formula feeds (50% full intake on d of life (DOL 2 (EA or 5 (LA while PN continued. Pigs were also fed formula containing either intact or hydrolyzed protein. In Experiment 2, preterm pigs received TPN at birth with enteral, hydrolyzed-protein formula feeds introduced on DOL 2 either abruptly (EA; 50% full feeds or gradually (EG; 10-50% full feeds over 5 d while PN continued. NEC incidence and severity were assessed based on macroscopic and histological scoring. In Experiment 1, NEC incidence (41% vs. 70%, P<0.05 and severity were reduced in LA vs. EA groups and LA was associated with a higher survival rate, daily weight gain and jejunum villus height. Piglets fed hydrolyzed vs. intact protein formula had lower stomach content weights and similar NEC incidence. In Experiment 2, NEC incidence and severity were not different between pigs the EG vs. EA group. Proinflammatory gene expression (IL-1β, IL-6 and S100A9 in the ileum was lower in both LA and EG vs. EA groups. In conclusion, delayed initiation but not gradual advancement of enteral feeding is protective against NEC in preterm pigs. Feeding hydrolyzed vs. intact protein formula improved gastric transit without affecting the NEC incidence.

28 CFR 345.59 - Inmate performance pay.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Inmate performance pay. 345.59 Section... INDUSTRIES (FPI) INMATE WORK PROGRAMS Inmate Pay and Benefits § 345.59 Inmate performance pay. Inmate workers for FPI may also receive Inmate Performance Pay for participation in programs where this award is made...

Syndromes with supernumerary teeth.

Science.gov (United States)

Lubinsky, Mark; Kantaputra, Piranit Nik

2016-10-01

While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

What Is Usher Syndrome?

Science.gov (United States)

... Action You are here Home › Retinal Diseases Listen Usher Syndrome What is Usher syndrome? How is Usher syndrome ... available? Are there any related diseases? What is Usher Syndrome? Usher syndrome is an inherited condition characterized by ...

Morphology and electrical properties of electrochemically synthesized pyrrole–formyl pyrrole copolymer

Energy Technology Data Exchange (ETDEWEB)

Gholami, Mehrdad, E-mail: mehrdad897@um.edu.my [Department of Chemistry, University of Malaya, Kuala Lumpur 50603 (Malaysia); Department of Chemistry, Marvdasht Branch, Islamic Azad University, P.O. Box 465, Marvdasht (Iran, Islamic Republic of); Nia, Pooria Moozarm, E-mail: pooriamn@yahoo.com [Department of Chemistry, University of Malaya, Kuala Lumpur 50603 (Malaysia); Alias, Yatimah, E-mail: yatimah70@um.edu.my [Department of Chemistry, University of Malaya, Kuala Lumpur 50603 (Malaysia)

2015-12-01

Graphical abstract: - Highlights: • The (Py–co-FPy) copolymer was synthesized electrochemically. • This copolymer has 1.6 times higher surface coverage compared to polypyrrole. • This copolymer showed 2.5 times lower resistance compared to polypyrrole. • The conjugated structure between Py and FPy causes enhancement of conductivity. • This conducting copolymer has a strong potential to be used in various applications. - Abstract: A direct electrochemical copolymerization of pyrrole–formyl pyrrole (Py–co-FPy) was carried out by oxidative copolymerization of formyl pyrrole and pyrrole in LiClO{sub 4} aqueous solution through galvanostatic method. The (Py–co-FPy) copolymer was characterized using Fourier-transform infrared spectroscopy (FT-IR), field emission scanning electron microscope (FESEM), energy-filtering transmission electron microscope (EFTEM), thermal gravimetric analysis (TGA), X-ray photoelectron spectroscopy (XPS), cyclic voltammetry (CV) and electrochemical impedance spectroscopy (EIS). The FESEM images showed that the synthesized copolymer had a hollow whelk-like helixes structure, which justifies the enhancement of charge transportation through the copolymer film. Cyclic voltammetry studies revealed that the electrocatalytic activity of synthesized copolymer has improved and the surface coverage in copolymer enhanced 1.6 times compared to polypyrrole alone. Besides, (Py–co-FPy) copolymer showed 2.5 times lower electrochemical charge transfer resistance (R{sub ct}) value in impedance spectroscopy. Therefore, this copolymer has a strong potential to be used in several applications such as sensor applications.

INFLUENCE OF FETOPLACENTAL INSUFFICIENCY ON THE FORMATION OF PERINATAL PATHOLOGY IN PRETERM NEONATES

Directory of Open Access Journals (Sweden)

N. P. Molokanova

2013-01-01

Full Text Available Aim: to identify features of early neonatal period in preterm infants of women with fetoplacental insufficiency (FPI. Patients and methods: we conducted a retrospective study of early neonatal period in 76 preterm infants with gestational age from 28 to 36 weeks. Of these, 31 were born by women with FPI (study group and 45 neonates from mothers without complications during pregnancy (control group. We analyzed course of pregnancy and delivery in both groups. Infant’s conditions were assessed using Apgar and Silverman Score. Neurological status, severity of respiratory and cardio-vascular failure were take into account in the first 7 days of life. Results: women with FPI had significantly higher levels of total extragenital pathology, hypertension was found only in mothers of the main group. The extent of Doppler abnormalities in most cases was light, while the most severe 3d grade was detected only in 4 women (12,9%, which had caused the birth of infants in severe asphyxia. Pathology of respiratory and central nervous system was dominated in preterm infants of both groups. Conclusions: the number of infants with serious conditions born by mothers with FPI is significantly higher than that determined by the deterioration of the utero-placental blood flow. Preterm neonates in women with FPI form a risk group of perinatal asphyxia.

28 CFR 345.56 - Vacation pay.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Vacation pay. 345.56 Section 345.56... (FPI) INMATE WORK PROGRAMS Inmate Pay and Benefits § 345.56 Vacation pay. Inmate workers are granted FPI vacation pay by the SOI when their continued good work performance justifies such pay, based on...

28 CFR 345.58 - Holiday pay.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Holiday pay. 345.58 Section 345.58... (FPI) INMATE WORK PROGRAMS Inmate Pay and Benefits § 345.58 Holiday pay. An inmate worker in FPI work status shall receive pay at the standard hourly rate, plus longevity where applicable, for all Federal...

Facilities Performance Indicators Report, 2006-07

Science.gov (United States)

Glazner, Steve, Ed.

2008-01-01

The "Facilities Performance Indicators Survey" ("FPI") supersedes and builds upon the two major surveys APPA conducted in the past: the Comparative Costs and Staffing (CCAS) survey and the Strategic Assessment Model (SAM). The "FPI" covers all the materials collected in CCAS and SAM, along with some select new data points and improved survey…

Autoimmune/inflammatory syndrome induced by adjuvants (Shoenfeld's syndrome) - An update.

Science.gov (United States)

Watad, A; Quaresma, M; Brown, S; Cohen Tervaert, J W; Rodríguez-Pint, I; Cervera, R; Perricone, C; Shoenfeld, Y

2017-06-01

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been widely described in many studies conducted thus far. The syndrome incorporates five immune-mediated conditions, all associated with previous exposure to various agents such as vaccines, silicone implants and several others. The emergence of ASIA syndrome is associated with individual genetic predisposition, for instance those carrying HLA-DRB1*01 or HLA-DRB4 and results from exposure to external or endogenous factors triggering autoimmunity. Such factors have been demonstrated as able to induce autoimmunity in both animal models and humans via a variety of proposed mechanisms. In recent years, physicians have become more aware of the existence of ASIA syndrome and the relationship between adjuvants exposure and autoimmunity and more cases are being reported. Accordingly, we have created a registry that includes at present more than 300 ASIA syndrome cases that have been reported by different physicians worldwide, describing various autoimmune conditions induced by diverse adjuvants. In this review, we have summarized the updated literature on ASIA syndrome and the knowledge accumulated since 2013 in order to elucidate the association between the exposure to various adjuvant agents and its possible clinical manifestations. Furthermore, we especially referred to the relationship between ASIA syndrome and systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS).

Toxic shock syndrome

Science.gov (United States)

Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

Eosinophilic leukocytoclastic vasculitis - a spectrum ranging from Wells' syndrome to Churg-Strauss syndrome?

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Ratzinger, Gudrun; Zankl, Julia; Eisendle, Klaus; Zelger, Bernhard

2014-01-01

Wells' syndrome is defined as an inflammatory disorder with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Eosinophilic leukocytoclastic vasculitis shows eosinophilic infiltrates in combination with vasculitic changes. And Churg Strauss Syndrome comprises all three characteristics - eosinophilic infiltrates, vasculitis and flame figures. To determine whether these three diseases are distinct entities or different manifestations of a similar clinicopathologic process. Histopathological samples and clinical courses of 17 patients with eosinophilic infiltrates, flame figures and clinical features of Wells' syndrome were re-evaluated. Histopathologically, we focused on the presence or absence of vasculitic features. Clinically, we included only patients who were diagnosed with Wells' syndrome at least once in the course of their disease. 4 patients were finally diagnosed with Wells' syndrome, 5 with eosinophilic leukocytoclastic vasculitis and 6 with Churg Strauss syndrome. Further, we had one case of an overlap between Wells' syndrome and eosinophilic vasculitis and one case of Wegener granulomatosis. Vasculitic features were found in the samples of all patients. Histologically, we find vasculitic features in typical presentations of Wells' syndrome. Clinically, we find typical features of Wells' syndrome in patients finally diagnosed with eosinophilic leukocytoclastic vasculitis or Churg Strauss syndrome. Furthermore, we have observed and formerly reported 3 patients with progression from Wells' syndrome to Churg Strauss syndrome. Thus, we assume that eosinophilic leukocytoclastic vasculitis might form a bridge between Wells' syndrome and Churg Strauss syndrome.

Management of moyamoya syndrome in patients with Noonan syndrome.

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Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

2016-06-01

A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

Obstructive sleep apnoea/hypopnoea syndrome in adults with Down syndrome

OpenAIRE

Hill, Elizabeth A.

2016-01-01

Key points Adults with Down syndrome are predisposed to obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to overlap between the Down syndrome phenotype and OSAHS risk factors. The prevalence of OSAHS in adults with Down syndrome is estimated at 35?42%. This is up to ten-times higher than in the general adult population. Symptoms of OSAHS, including behavioural and emotional disturbances as well as standard symptoms such as sleepiness, should be monitored as part of regular health surve...

Overweight, obesity and foot posture in children: A cross-sectional study.

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Gijon-Nogueron, Gabriel; Montes-Alguacil, Jesus; Martinez-Nova, Alfonso; Alfageme-Garcia, Pilar; Cervera-Marin, Jose A; Morales-Asencio, Jose M

2017-01-01

The aim of this study is to examine the relationship between obesity and foot posture in children. This cross-sectional study is based on a sample population of 1798 schoolchildren (873 boys and 925 girls) aged between 6 and 12 years. The height and weight of each subject was measured and the body mass index (BMI) was calculated. Foot posture was described by means of the foot posture index (FPI). The differences among various foot postures in relation to BMI, for the total sample, were tested using the Games-Howell test. In addition, cross tabulation for different gender groups and BMI categories was applied and tested using χ 2 . The mean BMI was 18.94 (standard deviation (SD) 3.65 kg/m 2 ) in the boys and 18.90 (SD 3.64 kg/m 2 ) in the girls, and the FPI was 3.97 (SD 2.98) in the boys and 3.68 (SD 2.86) in the girls. The FPI results show that among the boys aged 6 years, the right foot was more pronated than among the girls (FPI 4.8-4.1, P = 0.034), while among the boys aged 7 years, this was true for the left foot (4.4-3.7, P = 0.049). For the other ages, there were no significant differences in the FPI between the sexes. There were no significant differences between the value, or categories, of BMI and the FPI in the different age groups. In children aged between 6 and 12 years, body mass does not appear to have an important bearing on static foot posture. Furthermore, the variables gender and age are of scant importance in determining foot posture in children. © 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).

Examination of the Correlation Between Foot Morphology Measurements Using Pedography and Radiographic Measurements.

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Inui, Kentaro; Ikoma, Kazuya; Imai, Kan; Ohashi, Suzuyo; Maki, Masahiro; Kido, Masamitsu; Hara, Yusuke; Oka, Yoshinobu; Fujiwara, Hiroyoshi; Kubo, Toshikazu

Pedography provides excellent visualization of the footprint. However, the correlation between the footprint images and radiographic measures has not been thoroughly evaluated. Therefore, the objectives of our study were to examine the correlation between the pedography-based measures of foot morphology and radiographic measurements and to propose reference values for the diagnosis of flatfoot using footprint imaging. The plantar footprints of 100 right feet were photographed using a pedography standing platform. The sole and arch areas were measured to calculate the footprint index (FPI). The lateral talar-first metatarsal angle (LTM) and calcaneal pitch angle (CP) were measured on standing lateral radiographs, and the talonavicular coverage angle was measured on frontal radiographs. The Pearson moment correlation between the FPI and radiography-based measures was calculated. The area under the receiver operating characteristic curve was calculated using an LTM of <-4° as the identifying criterion of flatfoot. The sensitivity and specificity of FPI were calculated for LTM values <-4°. The FPI correlated with the LTM (y = -17.964 ± 52.644x, R = 0.588) and CP (y = 9.2304 ± 27.739x, R = 0.659) but not with the talonavicular coverage angle (y = 26.01 ± 15.78x, R = 0.207). The area under the receiver operating characteristic curve was 0.753, with a cutoff FPI of 0.208, yielding a sensitivity of 0.462 and specificity of 0.934 for flatfoot identification. Pedography could provide an easy screening tool for flatfoot, with an FPI cutoff of 0.208, yielding a specificity of 93.4%. Copyright © 2016 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

Contributions of foot muscles and plantar fascia morphology to foot posture.

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Angin, Salih; Mickle, Karen J; Nester, Christopher J

2018-03-01

The plantar foot muscles and plantar fascia differ between different foot postures. However, how each individual plantar structure contribute to foot posture has not been explored. The purpose of this study was to investigate the associations between static foot posture and morphology of plantar foot muscles and plantar fascia and thus the contributions of these structures to static foot posture. A total of 111 participants were recruited, 43 were classified as having pes planus and 68 as having normal foot posture using Foot Posture Index assessment tool. Images from the flexor digitorum longus (FDL), flexor hallucis longus (FHL), peroneus longus and brevis (PER), flexor hallucis brevis (FHB), flexor digitorum brevis (FDB) and abductor hallucis (AbH) muscles, and the calcaneal (PF1), middle (PF2) and metatarsal (PF3) regions of the plantar fascia were obtained using a Venue 40 ultrasound system with a 5-13 MHz transducer. In order of decreasing contribution, PF3 > FHB > FHL > PER > FDB were all associated with FPI and able to explain 69% of the change in FPI scores. PF3 was the highest contributor explaining 52% of increases in FPI score. Decreased thickness was associated with increased FPI score. Smaller cross sectional area (CSA) in FHB and PER muscles explained 20% and 8% of increase in FPI score. Larger CSA of FDB and FHL muscles explained 4% and 14% increase in FPI score respectively. The medial plantar structures and the plantar fascia appear to be the major contributors to static foot posture. Elucidating the individual contribution of multiple muscles of the foot could provide insight about their role in the foot posture. Copyright © 2018. Published by Elsevier B.V.

Lateral Fluid Percussion Injury Impairs Hippocampal Synaptic Soluble N-Ethylmaleimide Sensitive Factor Attachment Protein Receptor Complex Formation

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Shaun W. Carlson

2017-10-01

Full Text Available Traumatic brain injury (TBI and the activation of secondary injury mechanisms have been linked to impaired cognitive function, which, as observed in TBI patients and animal models, can persist for months and years following the initial injury. Impairments in neurotransmission have been well documented in experimental models of TBI, but the mechanisms underlying this dysfunction are poorly understood. Formation of the soluble N-ethylmaleimide-sensitive factor attachment protein receptor (SNARE complex facilitates vesicular docking and neurotransmitter release in the synaptic cleft. Published studies highlight a direct link between reduced SNARE complex formation and impairments in neurotransmitter release. While alterations in the SNARE complex have been described following severe focal TBI, it is not known if deficits in SNARE complex formation manifest in a model with reduced severity. We hypothesized that lateral fluid percussion injury (lFPI reduces the abundance of SNARE proteins, impairs SNARE complex formation, and contributes to impaired neurobehavioral function. To this end, rats were subjected to lFPI or sham injury and tested for acute motor performance and cognitive function at 3 weeks post-injury. lFPI resulted in motor impairment between 1 and 5 days post-injury. Spatial acquisition and spatial memory, as assessed by the Morris water maze, were significantly impaired at 3 weeks after lFPI. To examine the effect of lFPI on synaptic SNARE complex formation in the injured hippocampus, a separate cohort of rats was generated and brains processed to evaluate hippocampal synaptosomal-enriched lysates at 1 week post-injury. lFPI resulted in a significant reduction in multiple monomeric SNARE proteins, including VAMP2, and α-synuclein, and SNARE complex abundance. The findings in this study are consistent with our previously published observations suggesting that impairments in hippocampal SNARE complex formation may contribute to

Interferometric filters for spectral discrimination in high-spectral-resolution lidar: performance comparisons between Fabry-Perot interferometer and field-widened Michelson interferometer.

Science.gov (United States)

Cheng, Zhongtao; Liu, Dong; Yang, Yongying; Yang, Liming; Huang, Hanlu

2013-11-10

Thanks to wavelength flexibility, interferometric filters such as Fabry-Perot interferometers (FPIs) and field-widened Michelson interferometers (FWMIs) have shown great convenience for spectrally separating the molecule and aerosol scattering components in the high-spectral-resolution lidar (HSRL) return signal. In this paper, performance comparisons between the FPI and FWMI as a spectroscopic discrimination filter in HSRL are performed. We first present a theoretical method for spectral transmission analysis and quantitative evaluation on the spectral discrimination. Then the process in determining the parameters of the FPI and FWMI for the performance comparisons is described. The influences from the incident field of view (FOV), the cumulative wavefront error induced by practical imperfections, and the frequency locking error on the spectral discrimination performance of the two filters are discussed in detail. Quantitative analyses demonstrate that FPI can produce higher transmittance while the remarkable spectral discrimination is one of the most appealing advantages of FWMI. As a result of the field-widened design, the FWMI still performs well even under the illumination with large FOV while the FPI is only qualified for a small incident angle. The cumulative wavefront error attaches a great effect on the spectral discrimination performance of the interferometric filters. We suggest if a cumulative wavefront error is less than 0.05 waves RMS, it is beneficial to employ the FWMI; otherwise, FPI may be more proper. Although the FWMI shows much more sensitivity to the frequency locking error, it can outperform the FPI given a locking error less than 0.1 GHz is achieved. In summary, the FWMI is very competent in HSRL applications if these practical engineering and control problems can be solved, theoretically. Some other estimations neglected in this paper can also be carried out through the analytical method illustrated herein.

28 CFR 345.55 - Longevity pay.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Longevity pay. 345.55 Section 345.55... (FPI) INMATE WORK PROGRAMS Inmate Pay and Benefits § 345.55 Longevity pay. (a) Except as provided in paragraph (b) of this section, an inmate earns longevity pay raises after 18 months spent in FPI work status...

What is Metabolic Syndrome?

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... Intramural Research Home / Metabolic Syndrome Metabolic Syndrome Also known as What Is Metabolic syndrome ... metabolic risk factors to be diagnosed with metabolic syndrome. Metabolic Risk Factors A Large Waistline Having a large ...

Prune belly syndrome

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Eagle-Barrett syndrome; Triad syndrome ... The exact causes of prune belly syndrome are unknown. The condition affects mostly boys. While in the womb, the developing baby's abdomen swells with fluid. Often, the cause is ...

Hypersonic force measurements using internal balance based on optical micromachined Fabry-Perot interferometry

Science.gov (United States)

Qiu, Huacheng; Min, Fu; Zhong, Shaolong; Song, Xin; Yang, Yanguang

2018-03-01

Force measurements using wind tunnel balance are necessary for determining a variety of aerodynamic performance parameters, while the harsh environment in hypersonic flows requires that the measurement instrument should be reliable and robust, in against strong electromagnetic interference, high vacuum, or metal (oxide) dusts. In this paper, we demonstrated a three-component internal balance for hypersonic aerodynamic force measurements, using novel optical micromachined Fabry-Perot interferometric (FPI) strain gauges as sensing elements. The FPI gauges were fabricated using Micro-Opto-Electro-Mechanical Systems (MOEMS) surface and bulk fabrication techniques. High-reflectivity coatings are used to form a high-finesse Fabry-Perot cavity, which benefits a high resolution. Antireflective and passivation coatings are used to reduce unwanted interferences. The FPI strain gauge based balance has been calibrated and evaluated in a Mach 5 hypersonic flow. The results are compared with the traditional technique using the foil resistive strain gauge balance, indicating that the proposed balance based on the MOEMS FPI strain gauge is reliable and robust and is potentially suitable for the hypersonic wind tunnel harsh environment.

Gorlin-goltz syndrome

Directory of Open Access Journals (Sweden)

B V Shobha

2011-01-01

Full Text Available Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.

An incidental enterocolic lymphocytic phlebitis pattern is seen commonly in the rectal stump of patients with diversion colitis superimposed on inflammatory bowel disease.

Science.gov (United States)

Chetty, R; Hafezi, S; Montgomery, E

2009-05-01

Enterocolic lymphocytic phlebitis (ELP) is an uncommon cause of bowel pathology and most frequently results in ischaemia. It is characterised by an artery-sparing, venulocentric lymphoid infiltrate that causes a phlebitis and vascular compromise. Rare cases of ELP have been encountered with lymphocytic colitis in the absence of ischaemic bowel change. The present study examined the occurrence of ELP in the setting of diversion colitis and inflammatory bowel disease, as well as in random colectomy specimens. The study cohort comprised the following: 26 completion proctectomy specimens for ulcerative colitis with superimposed diversion colitis in the rectal stump; 3 colectomy specimens for Crohn disease with diversion colitis; 6 colectomy specimens for adenocarcinoma and/or diverticular disease with diversion colitis; 34 resection specimens with ulcerative colitis only; 19 with Crohn disease only; and 100 random colon resection specimens for adenocarcinoma, adenoma, diverticular disease and ischaemia. ELP was present in 18 of the 26 ulcerative colitis cases with diversion colitis, 3/3 Crohn disease cases with diversion colitis, 1/6 cases of diverticular disease with diversion colitis, 6/34 cases of ulcerative colitis without diversion, 2/19 Crohn disease cases without diversion colitis, and only 1 of 100 colectomy cases without inflammatory bowel disease or diversion colitis. ELP occurs most frequently in cases that have been diverted for inflammatory bowel disease. Fewer cases of ELP were noted in cases of inflammatory bowel disease in the absence of diversion colitis. It is postulated that altered bowel flora and immune dysregulation may be pivotal in the causation of this association.

Refeeding syndrome

OpenAIRE

Tripathy, Swagata; Mishra, Padmini; Dash, S. C.

2008-01-01

Refeeding syndrome is a potentially fatal medical condition that may affect malnourished patients in response to an inappropriately rapid overfeeding. This commonly occurs following the institution of nutritional support, especially parenteral or enteral nutrition. The most characteristic pathophysiology of refeeding syndrome relates to the rapid consumption of phosphate after glucose intake and subsequent hypophosphatemia. Refeeding syndrome can manifest as either metabolic changes （hypokala...

Nevoid basal cell carcinoma syndrome

Science.gov (United States)

NBCC syndrome; Gorlin-Goltz syndrome; Basal cell nevus syndrome; BCNS; Basal cell cancer - nevoid basal cell carcinoma syndrome ... Nevoid basal cell carcinoma nevus syndrome is a rare genetic ... syndrome is known as PTCH ("patched"). The gene is passed down ...

The wellness syndrome

DEFF Research Database (Denmark)

Mik-Meyer, Nanna

2015-01-01

Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.).......Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.)....

Turner Syndrome: Other FAQs

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... Other FAQs Share Facebook Twitter Pinterest Email Print Turner Syndrome: Other FAQs Basic information for topics, such as " ... been diagnosed with Turner syndrome. Now what? Is Turner syndrome inherited? Turner syndrome is usually not inherited, but ...

Bardet-Biedl syndrome and Usher syndrome.

Science.gov (United States)

Koenig, Rainer

2003-01-01

Bardet-Biedl syndrome (BBS) and Usher syndrome (USH) are the most prevalent syndromic forms of retinitis pigmentosa (RP), together they make up almost a quarter of the patients with RP. BBS is defined by the association of retinopathy, obesity, hypogonadism, renal dysfunction, postaxial polydactyly and mental retardation. This clinically complex syndrome is genetically heterogeneous with linkage to more than 6 loci, and 4 genes have been cloned so far. Recent molecular data present evidence that, in some instances, the clinical manifestation of BBS requires recessive mutations in 1 of the 6 BBS loci plus one or two additional mutations in a second BBS locus (tri- or tetra-allelic inheritance). USH is characterized by the combination of congenital or early-onset sensorineural deafness, RP, and variable degrees of vestibular dysfunction. Each of the three clinical types is genetically heterogeneous: 7 loci have been mapped for type 1, three loci for type 2, and two loci for type 3. Currently, 6 USH genes (MYO7A, USH1C, CDH23, PCDH15, USH2A, USH3) have been identified. Pathogenetically, mutations of the USH1 genes seem to result in defects of auditory and retinal sensory cells, the USH 2 phenotype is caused by defects of extracellular matrix or cell surface receptor proteins, and USH3 may be due to synaptic disturbances. The considerable contribution of syndromic forms of RP requires interdisciplinary approaches to the clinical and diagnostic management of RP patients.

Abdominal compartment syndrome with acute reperfusion syndrome

International Nuclear Information System (INIS)

Maleeva, A.

2017-01-01

Abdominal compartment syndrome was recognized clinically in the 19th century when Marey and Burt observed its association with declines in respiratory function. Abdominal compartment syndrome is first used as a medical terminology from Fietsman in a case of ruptured abdominal aortic aneurysm. A condition caused by abnormally increased pressure within the abdomen. Causes of abdominal compartment syndrome include trauma, surgery, or infection. Common symptoms: abdominal distension, fast heart rate, insufficient urine production, or low blood pressure Medical procedure: nasogastric intubation Surgery: laparotomy Specialists: radiologist, primary care provider (PCP), surgeon, and emergency medicine doctor [6, 10]. Keywords: Stomach. Gastroparesis . Diabetes Mellitus [bg

Munchausen syndrome and Munchausen syndrome by proxy in dermatology.

Science.gov (United States)

Boyd, Alan S; Ritchie, Coleman; Likhari, Sunaina

2014-08-01

Patients with Munchausen syndrome purposefully injure themselves, often with the injection of foreign materials, to gain hospital admission and the attention associated with having a difficult-to-identify condition. Munchausen syndrome by proxy occurs when a child's caregiver, typically the mother, injures the child for the same reasons. Cases of Munchausen syndrome and Munchausen syndrome by proxy with primary cutaneous involvement appear to be rarely described in the literature suggesting either that diagnosis is not made readily or that it is, in fact, an uncommon disorder. At the center of both conditions is significant psychological pathology and treatment is difficult as many patients with Munchausen syndrome when confronted with these diagnostic possibilities simply leave the hospital. Little is known about the long-term outcome or prognosis of these patients. Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

Roberts-SC syndrome, a rare syndrome and cleft palate repair

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Murthy Jyotsna

2008-01-01

Full Text Available Roberts SC syndrome is a rare syndrome with only 17 previously recognized patients reported in medical literature. The syndrome is characterized by multiple malformations, particularly, symmetrical limb reduction, craniofacial anomalies such as bilateral cleft lip and palate, micrognathia, and severe growth and mental retardation. Our patient, a young child of five years having Roberts-SC, was successfully operated for cleft palate under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed in this report.

Sjogren-Larsson Syndrome

Science.gov (United States)

... Or In Memory Of Obituaries Contact Us Donate Sjogren-Larsson Syndrome What causes SLS? SLS is caused by mutations ... methods of diagnosing SLS. Other Clinical Names for Sjogren-Larsson Syndrome Other clinical names of Sjogren-Larsson Syndrome include: ...

Redefining syndromic surveillance

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Rebecca Katz

2011-12-01

Full Text Available With growing concerns about international spread of disease and expanding use of early disease detection surveillance methods, the field of syndromic surveillance has received increased attention over the last decade. The purpose of this article is to clarify the various meanings that have been assigned to the term syndromic surveillance and to propose a refined categorization of the characteristics of these systems. Existing literature and conference proceedings were examined on syndromic surveillance from 1998 to 2010, focusing on low- and middle-income settings. Based on the 36 unique definitions of syndromic surveillance found in the literature, five commonly accepted principles of syndromic surveillance systems were identified, as well as two fundamental categories: specific and non-specific disease detection. Ultimately, the proposed categorization of syndromic surveillance distinguishes between systems that focus on detecting defined syndromes or outcomes of interest and those that aim to uncover non-specific trends that suggest an outbreak may be occurring. By providing an accurate and comprehensive picture of this field’s capabilities, and differentiating among system types, a unified understanding of the syndromic surveillance field can be developed, encouraging the adoption, investment in, and implementation of these systems in settings that need bolstered surveillance capacity, particularly low- and middle-income countries.

28 CFR 345.51 - Inmate pay.

Science.gov (United States)

2010-07-01

... 28 Judicial Administration 2 2010-07-01 2010-07-01 false Inmate pay. 345.51 Section 345.51... (FPI) INMATE WORK PROGRAMS Inmate Pay and Benefits § 345.51 Inmate pay. (a) Grade levels. Inmate workers in FPI locations receive pay at five levels ranging from 5th grade pay (lowest) to 1st grade pay...

Gorlin-Goltz Syndrome

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Padma Pandeshwar

2012-01-01

Full Text Available The Gorlin-Goltz syndrome (GGS (the nevoid basal cell carcinoma syndrome—NBCCS is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multidisciplinary problem, early diagnosis of which allows introduction of secondary prophylaxis and following an appropriate treatment to delay the progress of the syndrome. The following report emphasizes the need for awareness of the diagnostic criteria of this syndrome in cases with no typical skin lesions.

Sjögren syndrome

Science.gov (United States)

Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome ... The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most ...

Marfan Syndrome (For Parents)

Science.gov (United States)

... en español Síndrome de Marfan What Is Marfan Syndrome? Marfan syndrome is a genetic disorder of the body's ... bones , blood vessels, and organs. What Causes Marfan Syndrome? Marfan syndrome happens because of an abnormality in one ...

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome).

Science.gov (United States)

Kiran, N K; Tilak Raj, T N; Mukunda, K S; Rajashekar Reddy, V

2012-10-01

The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome

Directory of Open Access Journals (Sweden)

N K Kiran

2012-01-01

Full Text Available The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS, is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

``Battered child`` syndrome; Das ``Battered-Child``-Syndrom

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Elsner, K.; Merk, J.; Sokiranski, R. [Ulm Univ. (Germany). Abt. Diagnostische Radiologie

1997-10-01

Synonyms for the `battered child` syndrome (BCS) are terms describing the physical and body aspects of the process, such as `child abuse`, or `non-accidental injury`. These are to be distinguished from the psychic aspects and abuse, emotional and bodily neglect, and sexual abuse. Most cases are one or another combination of these aspects. Radiology is the essential method for giving proof of such abuses, identifying the signs of maltreatment in a medical record, or for disproving suspected abuse. (orig./AJ) [Deutsch] Als Synonym fuer das `Battered-Child`-Syndrom (BCS) stehen die Begriffe der koerperlichen-/physikalischen-Kindesmisshandlung, im angelsaechsischen Sprachraum die Begriffe `Child Abuse` und `Nonaccidental Injury`. Vom Syndrom abzugrenzen sind die seelische Misshandlung, die seelische und koerperliche Vernachlaessigung, und der sexuelle Missbrauch. Kombinationsformen sind nicht selten. Bei der Diagnostik des Syndroms spielt die Radiologie eine entscheidende Rolle. So hilft der Einsatz adaequater Untersuchungsmethoden, den Tatbestand der Misshandlung zu identifizieren und zu dokumentieren, aber auch einen Verdacht zu widerlegen. (orig./AJ)

Hepatorenal syndrome

Science.gov (United States)

... 2016:chap 153. Nevah MI, Fallon MB. Hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and other systemic complications of liver disease. In: Feldman M, Friedman LS, Brandt LJ, ...

Prevalence of Burnout Syndrome in patients admitted with acute coronary syndrome.

Science.gov (United States)

Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

2015-03-01

Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp's Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

Influence of Particle Size in Talc Suppression by a Galactomannan Depressant

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Zhixiang Chen

2018-03-01

Full Text Available Flotation behavior of different sizes of particles may follow different trends. The influence of particle size in talc suppression by a depressant galactomannan was studied in this research. The flotation response and mechanism were examined by flotation tests, modified flotation rate constant and entrainment recovery calculation, laser particle size experiments, adsorption tests, and advancing contact angle measurement as well as scanning electron microscopy (SEM and energy dispersive X-ray spectrometry (EDS. The maximum recovery increased with particle size increases in the absence of galactomannan FPY (Fenugreek polysaccharide. The obviously suppressed effect was observed for the size fraction of −74 + 38 μm after reacting with FPY, but low efficiency was received for −38 μm and −10 μm, respectively. Laser particle size analysis indicated that the FPY has a certain function for the flocculation of fine particles. It is beneficial for reducing recovery by entrainment. EDS and advancing contact angle test results showed that the difference in contact angles probably is a result of genuine differences in the quantity of O and Mg bearing surface species, while the contact angle varied with particle size fraction in the absence of FPY. Adsorption and SEM test results demonstrated that in the case of −74 + 38 μm, the depressant adsorption density on the mineral surface is higher than the other two size fractions. On the whole, FPY probably is not enough of a depressant for talc suppression.

Turner Syndrome (For Teens)

Science.gov (United States)

... Staying Safe Videos for Educators Search English Español Turner Syndrome KidsHealth / For Teens / Turner Syndrome What's in this ... en español El síndrome de Turner What Is Turner Syndrome? Turner syndrome (TS) is a genetic condition found ...

DIDMOAD (Wolfram Syndrome

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Masoud Nashibi

2016-07-01

Full Text Available Wolfram syndrome was first described by physician D J Wolfram and Wagener in 1938. This autosomal recessive syndrome is also referred to as DIDMOAD syndrome which stands for Diabetes Insipidus, Insulin Dependent Diabetes Mellitus, Optic Atrophy and Deafness

[The Capgras syndrome].

Science.gov (United States)

Anikina, M A; Levin, O S

2013-01-01

The Capgras syndrome is one of delusional-like misidentification syndrome in which a person holds a delusion that one or several his/her friends or relatives have been replaced by an identical-looking impostor. As any other delusional disorder, the Capgras syndrome is characterized by stability despite the indisputable arguments against fault views. Initially, this syndrome was considered as a presentation of schizophrenia but later it has been described in brain organic disorders, primarily in elderly patients with dementia.

Revesz syndrome

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Dayane Cristine Issaho

2015-04-01

Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

Salmonella enterocolitis

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... a hospital, nursing home, or other long-term health facility Have a pet iguana or other lizards, turtles, or snakes (reptiles ... Your health care provider will perform a physical exam. You may ... abdomen and tiny pink spots, called rose spots, on your skin. ...

Necrotizing Enterocolitis

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... large section of the intestine was removed, an ostomy is done. During an ostomy, surgeons bring an area of the intestine to ... to re-examine the intestines or close the ostomy. What Can I Expect? Most babies who develop ...

The Mediterranean diet adherence by pregnant women delivering prematurely: association with size at birth and complications of prematurity.

Science.gov (United States)

Parlapani, Elisavet; Agakidis, Charalampos; Karagiozoglou-Lampoudi, Thomais; Sarafidis, Kosmas; Agakidou, Eleni; Athanasiadis, Apostolos; Diamanti, Elisavet

2017-11-13

The Mediterranean diet (MD) is associated with decreased risk of metabolic syndrome and gestational diabetes due to the anti-inflammatory and antioxidative properties of its components. The aim was to investigate the potential association of MD adherence (MDA) during pregnancy by mothers delivering prematurely, with intrauterine growth as expressed by neonates' anthropometry at birth and complications of prematurity. This is a single-center, prospective, observational cohort study of 82 women who delivered preterm singletons at post conceptional age (PCA) ≤ 34 weeks and their live-born neonates. Maternal and neonatal demographic and clinical data were recorded. All mothers filled in a food frequency questionnaire, and the MDA score was calculated. Based on 50th centile of MD score, participants were classified into high-MDA and low-MDA groups. The low-MDA mothers had significantly higher pregestational BMI and rates of overweight/obesity (odd ratios (OR) 3.5) and gestational hypertension/preeclampsia (OR 3.8). Neonates in the low-MDA group had significantly higher incidence of intrauterine growth restriction (IUGR) (OR 3.3) and lower z-scores of birth weight and BMI. Regarding prematurity-related complications, the low MDA-group was more likely to develop necrotizing enterocolitis, bronchopulmonary dysplasia, and retinopathy of prematurity (OR 3.2, 1.3, and 1.6, respectively), while they were less likely to develop respiratory distress syndrome (OR 0.49), although the differences were not statistically significant. However, adjustment for confounders revealed MDA as a significant independent predictor of hypertension/preeclampsia, IUGR, birth weight z-score, necrotizing enterocolitis, and bronchopulmonary dysplasia. High MDA during pregnancy may favorably affect intrauterine growth and certain acute and chronic complications of prematurity as well as maternal hypertension/preeclampsia.

Cardiorenal Syndrome in Acute Heart Failure Syndromes

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Mohammad Sarraf

2011-01-01

Full Text Available Impaired cardiac function leads to activation of the neurohumoral axis, sodium and water retention, congestion and ultimately impaired kidney function. This sequence of events has been termed the Cardiorenal Syndrome. This is different from the increase in cardiovascular complications which occur with primary kidney disease, that is, the so-called Renocardiac Syndrome. The present review discusses the pathogenesis of the Cardiorenal Syndrome followed by the benefits and potential deleterious effects of pharmacological agents that have been used in this setting. The agents discussed are diuretics, aquaretics, natriuretic peptides, vasodilators, inotropes and adenosine α1 receptor antagonists. The potential role of ultrafiltration is also briefly discussed.

Interstitial Cystitis/Painful Bladder Syndrome and Associated Medical Conditions With an Emphasis on Irritable Bowel Syndrome, Fibromyalgia and Chronic Fatigue Syndrome

DEFF Research Database (Denmark)

Nickel, J.C.; Tripp, D.A.; Pontari, M.

2010-01-01

of associated conditions increased (ie localized, regional, systemic), pain, stress, depression and sleep disturbance increased while social support, sexual functioning and quality of life deteriorated. Anxiety and catastrophizing remained increased in all groups. Symptom duration was associated......Purpose: We characterized and compared the impact of clinical phenotypic associations between interstitial cystitis/painful bladder syndrome and controls in relation to potentially related conditions, particularly irritable bowel syndrome, fibromyalgia and chronic fatigue syndrome. Materials...... cystitis/painful bladder syndrome vs controls was irritable bowel syndrome 38.6% vs 5.2%, fibromyalgia 17.7% vs 2.6% and chronic fatigue syndrome 9.5% vs 1.7% (all p

Plummer-Vinson syndrome

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Novacek Gottfried

2006-09-01

Full Text Available Abstract Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. Etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

A randomized controlled trial of group Stepping Stones Triple P: a mixed-disability trial.

Science.gov (United States)

Roux, Gemma; Sofronoff, Kate; Sanders, Matthew

2013-09-01

Stepping Stones Triple P (SSTP) is a parenting program designed for families of a child with a disability. The current study involved a randomized controlled trial of Group Stepping Stones Triple P (GSSTP) for a mixed-disability group. Participants were 52 families of children diagnosed with an Autism Spectrum Disorder, Down syndrome, Cerebral Palsy, or an intellectual disability. The results demonstrated significant improvements in parent-reported child behavior, parenting styles, parental satisfaction, and conflict about parenting. Results among participants were similar despite children's differing impairments. The intervention effect was maintained at 6-month follow-up. The results indicate that GSSTP is a promising intervention for a mixed-disability group. Limitations of the study, along with areas for future research, are also discussed. © FPI, Inc.

Genetics Home Reference: antiphospholipid syndrome

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... Share: Email Facebook Twitter Home Health Conditions Antiphospholipid syndrome Antiphospholipid syndrome Printable PDF Open All Close All Enable ... area? Other Names for This Condition anti-phospholipid syndrome antiphospholipid antibody syndrome Hughes syndrome Related Information How are ...

Genetics Home Reference: Costello syndrome

Science.gov (United States)

... other genetic conditions, cardiofaciocutaneous syndrome (CFC syndrome) and Noonan syndrome . In affected infants, it can be difficult to ... These individuals may actually have CFC syndrome or Noonan syndrome , which are caused by mutations in related genes. ...

Metabolic syndrome and menopause

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Jouyandeh Zahra

2013-01-01

Full Text Available Abstract Background The metabolic syndrome is defined as an assemblage of risk factors for cardiovascular diseases, and menopause is associated with an increase in metabolic syndrome prevalence. The aim of this study was to assess the prevalence of metabolic syndrome and its components among postmenopausal women in Tehran, Iran. Methods In this cross-sectional study in menopause clinic in Tehran, 118 postmenopausal women were investigated. We used the adult treatment panel 3 (ATP3 criteria to classify subjects as having metabolic syndrome. Results Total prevalence of metabolic syndrome among our subjects was 30.1%. Waist circumference, HDL-cholesterol, fasting blood glucose, diastolic blood pressure ,Systolic blood pressure, and triglyceride were significantly higher among women with metabolic syndrome (P-value Conclusions Our study shows that postmenopausal status is associated with an increased risk of metabolic syndrome. Therefore, to prevent cardiovascular disease there is a need to evaluate metabolic syndrome and its components from the time of the menopause.

Wolfram syndrome 1 and Wolfram syndrome 2.

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Rigoli, Luciana; Di Bella, Chiara

2012-08-01

Wolfram syndrome 1 (WS1) is an autosomal recessive disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DI DM OA D syndrome) associated with other variable clinical manifestations. The causative gene for WS1 (WFS1) encoding wolframin maps to chromosome 4p16.1. Wolframin has an important function in maintaining the homeostasis of the endoplasmic reticulum (ER) in pancreatic β cells. Recently, another causative gene, CISD2, has been identified in patients with a type of Wolfram syndrome (WS2) resulting in early optic atrophy, diabetes mellitus, deafness, decreased lifespan, but not diabetes insipidus. The CISD2-encoded protein ERIS (endoplasmic reticulum intermembrane small protein) also localizes to ER, but does not interact directly with wolframin. ERIS maps to chromosome 4q22. Numerous studies have shown an interesting similarity between WFS1 and CISD2 genes. Experimental studies demonstrated that the Cisd2 knockout (Cisd2) mouse shows premature aging and typical symptoms of Wolfram syndrome. These researches provide interesting insight into the relation of neurodegenerative diseases, mitochondrial disorders, and autophagy and are useful for the pathophysiological understanding of both Wolfram syndrome and mitochondrial-mediated premature aging. The knowledge of WS1 and WS2 pathogenesis, and of the interactions between WFS1 and CISD2 genes, is useful for accurate diagnostic classification and for diagnosis of presymptomatic individuals.

Interaction Between Syndromic and Non-Syndromic Factors Affecting Speech and Language Development in Treacher-Collins Syndrome

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Marziyeh Poorjavad

2011-09-01

Full Text Available Background: Treacher-Collins syndrome is a congenital craniofacial disorder with multiple anomalies. This syndrome affects the maxilla, mandible, eyes, middle and outer ears, and soft palate. Conductive hearing loss due to the deformities of the middle and external ears is prevalent. The characteristics of this syndrome include multiple and serious threats to normal communication development in children. In this study, speech and language features of a Persian speaking child with this syndrome are presented.Case: The case was an 8-year old girl with Treacher-Collins syndrome and bilateral moderate conductive hearing loss due to atretic canal. In language and speech assessments, moderate hypernasality, numerous compensatory errors and morphosyntactic deficits were observed. There were 13 phonemes that were incorrectly produced at least in one position. Besides, she used 22 types of phonological processes that were abnormal and disappear before the age of three in normal Persian speaking children.Conclusion: Moderate hearing loss, velopharyngeal incompetency, malocclusion and dental anomalies, attention deficit/hyperactivity disorder (ADHD and environmental factors resulted in severe speech and language disorders in this case. These disorders affected her academic performance as well. Moderate hypernasality, numerous compensatory errors, and excessive and abnormal use of phonological processes were not presented as prevalent characteristics of Treacher-Collins syndrome in other resources.

Urofacial syndrome

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Kamal F Akl

2012-01-01

Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

Felty syndrome

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Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

Pendred Syndrome

Science.gov (United States)

... other possible long-term consequences of the syndrome. Children with Pendred syndrome should start early treatment to gain communication skills, such as learning sign language or cued speech or learning to ...

Unusual headache syndromes.

Science.gov (United States)

Queiroz, Luiz P

2013-01-01

Some headache syndromes have few cases reported in the literature. Their clinical characteristics, pathogenesis, and treatment may have not been completely defined. They may not actually be uncommon but rather under-recognized and/or underreported. A literature review of unusual headache syndromes, searching PubMed and ISI Web of Knowledge, was performed. After deciding which disorders to study, relevant publications in scientific journals, including original articles, reviews, meeting abstracts, and letters or correspondences to the editors were searched. This paper reviewed the clinical characteristics, the pathogenesis, the diagnosis, and the treatment of five interesting and unusual headache syndromes: exploding head syndrome, red ear syndrome, neck-tongue syndrome, nummular headache, and cardiac cephalgia. Recognizing some unusual headaches, either primary or secondary, may be a challenge for many non-headache specialist physicians. It is important to study them because the correct diagnosis may result in specific treatments that may improve the quality of life of these patients, and this can even be life saving. © 2013 American Headache Society.

Burnout Syndrome of Teachers

OpenAIRE

Semrádová, Michaela

2013-01-01

The bachelor's thesis covers burnout syndrome of teachers. Defines burnout syndrome, describes its causes and symptoms. Describes teaching as helping profession and focousing on stressful situations at school. In the last chapter described different prevention strategies burnout syndrome. Key words: burnout syndrome, teaching, teacher, helping professions, beginning teacher, stress

Prevalence of Burnout Syndrome in Patients Admitted with Acute Coronary Syndrome

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Ana Cláudia Giaxa Prosdócimo

2015-03-01

Full Text Available Background: Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS. Objective: To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods: Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI, which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE, emotional distancing (EmD, dehumanization (De and professional fulfillment (PF. The Lipp’s Stress Symptoms Inventory for Adults (LSSI was applied to evaluate global stress. Results: Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion: We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

"Nine" syndrome: A new neuro-ophthalmologic syndrome: Report of two cases

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Rohan R Mahale

2015-01-01

Full Text Available "Eight-and-a-half" syndrome is a rare condition involving the ipsilateral abducens nucleus or paramedian pontine reticular formation (PPRF, the ipsilateral medial longitudinal fasciculus (MLF, and the adjacent facial colliculus/facial nerve fascicle. The condition is often caused by a lesion (vascular or demyelinating in the dorsal tegmentum of the caudal pons. There are new variants of this syndrome caused by extension of lesion to involve new adjacent structures in pontine tegmentum. We report two patients with different etiology presenting with clinical features suggestive of eight-and-a-half syndrome associated with hemiataxia representing "nine" syndrome (8– + – = 9 adding new dimension to "eight-and-a-half" syndrome.

Mobius syndrome redefined: a syndrome of rhombencephalic maldevelopment.

NARCIS (Netherlands)

Verzijl, H.T.F.M.; Zwaag, A. van der; Cruysberg, J.R.M.; Padberg, G.W.A.M.

2003-01-01

OBJECTIVE: To investigate the variable clinical picture of Mobius syndrome (MIM no. 157900) and to further understand the pathogenesis of the disorder. METHODS: A standardized questionnaire was submitted to 37 Dutch patients with Mobius syndrome. All underwent standardized neurologic examination

Bartter syndrome

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... this page: //medlineplus.gov/ency/article/000308.htm Bartter syndrome To use the sharing features on this page, please enable JavaScript. Bartter syndrome is a group of rare conditions that affect ...

Dravet Syndrome

Science.gov (United States)

... and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome. Study of the genetic defects responsible for Dravet syndrome and related ... Publications Definition Dravet ...

Genetics Home Reference: Marfan syndrome

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... Share: Email Facebook Twitter Home Health Conditions Marfan syndrome Marfan syndrome Printable PDF Open All Close All Enable Javascript ... Marfan syndrome KidsHealth from Nemours Foundation MalaCards: marfan syndrome Orphanet: Marfan syndrome Your Genes Your Health from Cold Spring ...

Does wastewater discharge have relations with increase of Turner syndrome and Down syndrome?

Science.gov (United States)

Choi, Intae

2017-01-01

The purpose of this study is to examine whether water and air pollutants have a relationship with an increase in the genetic disorders Turner syndrome and Down syndrome, which are caused by congenital chromosomal abnormalities, and to generate a hypothesis about the genetic health effects of environmental pollutants. A panel regression based on random effect was conducted on Korea's metropolitan councils from 2012 to 2014. The dependent variable was the number of Turner syndrome and Down syndrome cases, and the main independent variables were those regarding the water and air pollution. Air pollutants did not have a significant impact on the number of Turner syndrome and Down syndrome cases; however, the increase in number of wastewater discharge companies did have a significant relationship with the number of cases. The more the number of wastewater discharge companies, the more the number Turner syndrome and Down syndrome cases were observed. Therefore, scientific investigation on water and air pollutants in relation with genetic health effects needs to be performed.

Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome: a case report

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Shukla Umesh

2010-11-01

Full Text Available Abstract Introduction We report the case of an unusual association of Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome. This association has never previously been reported in the medical literature. Case presentation A nine-year-old Indian girl was referred to our hospital for growth retardation, mental retardation, lax joints, generalized hypertrichosis, and hypoplastic fifth fingernails and toenails. A thorough medical examination and evaluation revealed she had phenotypic features of Coffin-Siris syndrome, with Mayer-Rokitansky-Küster-Hauser syndrome on radiological evaluation. The karyotype of our patient was normal. Conclusion In an unexplained case of mental retardation with facies suggestive of Coffin-Siris syndrome, association with Mayer-Rokitansky-Küster-Hauser syndrome should be considered and the patient should be evaluated for the same. Both of these syndromes may have a common pathogenesis, as yet unknown. This case report has broad implications, as similar cases in future may give insights into the pathogenesis of both these syndromes.

Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome: a case report.

Science.gov (United States)

Goyal, Deepak; Yadav, Dinesh K; Shukla, Umesh; Sethi, Sidharth K

2010-11-08

We report the case of an unusual association of Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome. This association has never previously been reported in the medical literature. A nine-year-old Indian girl was referred to our hospital for growth retardation, mental retardation, lax joints, generalized hypertrichosis, and hypoplastic fifth fingernails and toenails. A thorough medical examination and evaluation revealed she had phenotypic features of Coffin-Siris syndrome, with Mayer-Rokitansky-Küster-Hauser syndrome on radiological evaluation. The karyotype of our patient was normal. In an unexplained case of mental retardation with facies suggestive of Coffin-Siris syndrome, association with Mayer-Rokitansky-Küster-Hauser syndrome should be considered and the patient should be evaluated for the same. Both of these syndromes may have a common pathogenesis, as yet unknown. This case report has broad implications, as similar cases in future may give insights into the pathogenesis of both these syndromes.

The Mexican consensus on probiotics in gastroenterology

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M.A. Valdovinos

2017-04-01

Results and conclusions: Eleven statements on the general concepts of probiotics and 27 statements on the use of probiotics in gastrointestinal diseases in both adults and children were formulated. The consensus group recommends the use of probiotics under the following clinical conditions: the prevention of diarrhea associated with antibiotics, the treatment of acute infectious diarrhea, the prevention of Clostridium difficile infection and necrotizing enterocolitis, the reduction of adverse events from Helicobacter pylori eradication therapy, relief from irritable bowel syndrome symptoms, the treatment of functional constipation in the adult, and the induction and maintenance of remission in patients with ulcerative colitis (UC and pouchitis, and the treatment of covert and overt hepatic encephalopathy.

Rett Syndrome

Science.gov (United States)

Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

Caplan syndrome

Science.gov (United States)

... enable JavaScript. Rheumatoid pneumoconiosis (RP; also known as Caplan syndrome) is swelling (inflammation) and scarring of the ... avoid exposure to inorganic dust. Alternative Names RP; Caplan syndrome; Pneumoconiosis - rheumatoid; Silicosis - rheumatoid pneumoconiosis; Coal worker's ...

Sotos Syndrome

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... Clinical Trials Organizations Publications Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ... have also been reported. × Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ...

Chronic exertional compartment syndrome with medial tibial stress syndrome in twins.

Science.gov (United States)

Banerjee, Purnajyoti; McLean, Christopher

2011-06-14

Chronic exertional compartment syndrome and medial tibial stress syndrome are uncommon conditions that affect long-distance runners or players involved in team sports that require extensive running. We report 2 cases of bilateral chronic exertional compartment syndrome, with medial tibial stress syndrome in identical twins diagnosed with the use of a Kodiag monitor (B. Braun Medical, Sheffield, United Kingdom) fulfilling the modified diagnostic criteria for chronic exertional compartment syndrome as described by Pedowitz et al, which includes: (1) pre-exercise compartment pressure level >15 mm Hg; (2) 1 minute post-exercise pressure >30 mm Hg; and (3) 5 minutes post-exercise pressure >20 mm Hg in the presence of clinical features. Both patients were treated with bilateral anterior fasciotomies through minimal incision and deep posterior fasciotomies with tibial periosteal stripping performed through longer anteromedial incisions under direct vision followed by intensive physiotherapy resulting in complete symptomatic recovery. The etiology of chronic exertional compartment syndrome is not fully understood, but it is postulated abnormal increases in intramuscular pressure during exercise impair local perfusion, causing ischemic muscle pain. No familial predisposition has been reported to date. However, some authors have found that no significant difference exists in the relative perfusion, in patients, diagnosed with chronic exertional compartment syndrome. Magnetic resonance images of affected compartments have indicated that the pain is not due to ischemia, but rather from a disproportionate oxygen supply versus demand. We believe this is the first report of chronic exertional compartment syndrome with medial tibial stress syndrome in twins, raising the question of whether there is a genetic predisposition to the causation of these conditions. Copyright 2011, SLACK Incorporated.

Moyamoya syndrome in a patient with Noonan-like syndrome with loose anagen hair.

Science.gov (United States)

Choi, Jin-Ho; Oh, Moon-Yeon; Yum, Mi-Sun; Lee, Beom Hee; Kim, Gu-Hwan; Yoo, Han-Wook

2015-03-01

Noonan-like syndrome with loose anagen hair is one of the RASopathies characterized by Noonan syndrome-like features with unique ectodermal abnormalities. This syndrome is caused by mutations in the SHOC2 gene. We encountered a patient with moyamoya syndrome associated with Noonan-like syndrome with loose anagen hair presenting with transient ischemic attacks. A 6-year-old girl was diagnosed with Noonan-like syndrome with loose anagen hair because of profound short stature and ectodermal anomalies such as sparse and easily pluckable hair. A heterozygous mutation of c.4A>G (p.S2G) in the SHOC2 gene was identified, and recombinant human growth hormone therapy was initiated at 8 years of age. At age 10, she manifested recurrent left hemiplegia. Moreover, cerebrovascular imaging revealed occlusion or narrowing of both internal carotid arteries and both middle cerebral arteries with distal moyamoya-like vessels. She is treated with aspirin and calcium channel blocker. We describe the first case of Noonan-like syndrome with loose anagen hair associated with moyamoya syndrome, although it has been reported to be associated with a few cases of other RASopathies, including Noonan, cardiofaciocutaneous, and Costello syndromes. This report emphasizes the associations between cerebrovascular anomalies and Noonan-like syndrome with loose anagen hair. Copyright © 2015 Elsevier Inc. All rights reserved.

Abdominal vascular syndromes: characteristic imaging findings

International Nuclear Information System (INIS)

Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D'Ippolito, Giuseppe

2016-01-01

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

Abdominal vascular syndromes: characteristic imaging findings

Energy Technology Data Exchange (ETDEWEB)

Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D' Ippolito, Giuseppe, E-mail: leandrocleite@gmail.com [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Mediciana. Departmento de Diagnostico por Imagem

2016-07-15

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

Cushing's Syndrome

Science.gov (United States)

Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that ... your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

Usher Syndrome

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Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

Metabolic Syndrome

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Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These conditions ... agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

Reye Syndrome

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Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently ... a viral illness, seek medical attention immediately. Reye syndrome can lead to a coma and brain death, ...

Turner Syndrome

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Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

Apert syndrome (acrocephalosyndactyly

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Milovanović J.

2014-01-01

Full Text Available Apert syndrome is named for the French physician, Eugen Apert who was, in 1906. described anomalous shape of the skull with coronary suture synostosis and hypoplasia sphenoethmoidmaxillary part of the face and fingers syndactyly of hands and feet. Apert syndrome accounts for about 4,5% of all craniosynostosis. With the prevalence of 1:160 000-200 000, inherited in an autosomal domi­nant, and in 25% of cases are fresh mutations in the gene. This syndrome has no predilection by gender and race, varies in severity form in witch it is manifested. Anomality of internal organs are very rare, but half of the patients with this syndrome have mental retardation. Apert syndrome has no cure, but surgery can help to correct some of the problems.

Coexistent Brugada Syndrome and Wolff-Parkinson-White Syndrome: What is the Optimal Management?

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Abhishek Jaiswal, MBBS

2013-09-01

Full Text Available Coexistent Brugada syndrome and Wolff-Parkinson-White (WPW syndrome is rare, and as such poses management challenges. The overlap of symptoms attributable to each condition, the timing of ventricular stimulation after accessory pathway ablation and the predictive value of programmed stimulation in Brugada syndrome are controversial. We describe a case of coexistent Brugada syndrome and WPW syndrome in a symptomatic young adult. We discuss our treatment approach and the existing literature along with the challenges in management of such cases.

Cowden syndrome

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Ravi Prakash S

2010-01-01

Full Text Available Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

Metabolic syndrome and the risk of adverse cardiovascular events after an acute coronary syndrome.

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Cavallari, Ilaria; Cannon, Christopher P; Braunwald, Eugene; Goodrich, Erica L; Im, KyungAh; Lukas, Mary Ann; O'Donoghue, Michelle L

2018-05-01

Background The incremental prognostic value of assessing the metabolic syndrome has been disputed. Little is known regarding its prognostic value in patients after an acute coronary syndrome. Design and methods The presence of metabolic syndrome (2005 International Diabetes Federation) was assessed at baseline in SOLID-TIMI 52, a trial of patients within 30 days of acute coronary syndrome (median follow-up 2.5 years). The primary endpoint was major coronary events (coronary heart disease death, myocardial infarction or urgent coronary revascularization). Results At baseline, 61.6% ( n = 7537) of patients met the definition of metabolic syndrome, 34.7% (n = 4247) had diabetes and 29.3% had both ( n = 3584). The presence of metabolic syndrome was associated with increased risk of major coronary events (adjusted hazard ratio (adjHR) 1.29, p metabolic syndrome was numerically but not significantly associated with the risk of major coronary events (adjHR 1.13, p = 0.06). Conversely, diabetes was a strong independent predictor of major coronary events in the absence of metabolic syndrome (adjHR 1.57, p metabolic syndrome identified patients at highest risk of adverse outcomes but the incremental value of metabolic syndrome was not significant relative to diabetes alone (adjHR 1.07, p = 0.54). Conclusions After acute coronary syndrome, diabetes is a strong and independent predictor of adverse outcomes. Assessment of the metabolic syndrome provides only marginal incremental value once the presence or absence of diabetes is established.

Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea with Underlying Crohn’s Disease

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Е. А. Roslavtseva

2015-01-01

Full Text Available Tricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial features and hair growth abnormalities, immune disorders. There are data on two patients dealing with tricho-hepato-enteric syndrome with underlying Crohn’s disease. This is the first description of cases of aggravated tricho-hepatoenteric syndrome ever found in Russian medical literature. 

A new familial intrauterine growth retardation syndrome the "3-M syndrome".

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Spranger, J; Opitz, J M; Nourmand, A

1976-09-01

Two pairs of siblings are described with proportionate dwarfism due to skeletal hypoplasia of prenatal onset. The head size was normal for age and disproportionately large for height. The patients had a characteristic face different from that seen in the Silver-Russell syndrome. The family data are in accordance with autosomal recessive inheritance. In spite of some similarities, the bulk of clinical and genetic evidence suggests that the described intrauterine growth retardation syndrome is different from the Silver-Russell syndrome and presents an apparently "new" entity which has been designated 3-M syndrome.

Are ECG abnormalities in Noonan syndrome characteristic for the syndrome?

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Raaijmakers, R; Noordam, C; Noonan, J A; Croonen, E A; van der Burgt, C J A M; Draaisma, J M T

2008-12-01

Of all patients with Noonan syndrome, 50-90% have one or more congenital heart defects. The most frequent occurring are pulmonary stenosis (PS) and hypertrophic cardiomyopathy. The electrocardiogram (ECG) of a patient with Noonan syndrome often shows a characteristic pattern, with a left axis deviation, abnormal R/S ratio over the left precordium, and an abnormal Q wave. The objective of this study was to determine if these ECG characteristics are an independent feature of the Noonan syndrome or if they are related to the congenital heart defect. A cohort study was performed with 118 patients from two university hospitals in the United States and in The Netherlands. All patients were diagnosed with definite Noonan syndrome and had had an ECG and echocardiography. Sixty-nine patients (58%) had characteristic abnormalities of the ECG. In the patient group without a cardiac defect (n = 21), ten patients had a characteristic ECG abnormality. There was no statistical relationship between the presence of a characteristic ECG abnormality and the presence of a cardiac defect (p = 0.33). Patients with hypertrophic cardiomyopathy had more ECG abnormalities in total (p = 0.05), without correlation with a specific ECG abnormality. We conclude that the ECG features in patients with Noonan syndrome are characteristic for the syndrome and are not related to a specific cardiac defect. An ECG is very useful in the diagnosis of Noonan syndrome; every child with a Noonan phenotype should have an ECG and echocardiogram for evaluation.

In-line open-cavity Fabry-Pérot interferometer formed by C-shaped fiber fortemperature-insensitive refractive index sensing.

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Wu, Chuang; Liu, Zhengyong; Zhang, A Ping; Guan, Bai-Ou; Tam, Hwa-Yaw

2014-09-08

We report an open-cavity optical fiber Fabry-Pérot interferometer (FPI) capable of measuring refractive index with very low temperature cross-sensitivity. The FPI was constructed by splicing a thin piece of C-shaped fiber between two standard single-mode fibers. The refractive index (RI) response of the FPI was characterized using water-ethanol mixtures with RI in the range of 1.33 to 1.36. The RI sensitivity was measured to be 1368 nm/RIU at the wavelength of 1600 nm with good linearity. Thanks to its all-glass structure, the FPI exhibits very low temperature cross-sensitivity of 3.04 × 10⁻⁷ RIU/°C. The effects of cavity length on the performance of the sensor were also studied. A shorter cavity gives rise to broader measurement range while offering larger detection limit, and vice versa. What's more, the effect of material dispersion of analyte on the sensitivity of open-cavity FPIs was identified for the first time. The sensor is compact in size and easy to fabricate. It is potentially useful for label-free optical sensing of chemical and biological samples.

Brain-hepato-renal syndrome (Zellweger syndrome). Report of two cases and a review of the syndrome

International Nuclear Information System (INIS)

Ruiz, T.; Caparros, C.; Blanco, A.; Lopez, A.M.

1997-01-01

Cerebro-hepato-renal syndrome is a rare disorder that is transmitted by autosomal recessive inheritance. Children with this syndrome present mongoloid facies and severe muscle hypotonic at birth. Scimitar-like knee calcifications are considered a pathognomonic feature of this disorder. We present two patients with Zellweger syndrome, according to the diagnosis suggested by our Radiodiagnostic Service. Our objective is to stress the importance of the radiological findings, which in many cases are decisive in establishing the definitive diagnosis. (Author) 10 refs

Does wastewater discharge have relations with increase of Turner syndrome and Down syndrome?

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Intae Choi

2017-08-01

Full Text Available The purpose of this study is to examine whether water and air pollutants have a relationship with an increase in the genetic disorders Turner syndrome and Down syndrome, which are caused by congenital chromosomal abnormalities, and to generate a hypothesis about the genetic health effects of environmental pollutants. A panel regression based on random effect was conducted on Korea’s metropolitan councils from 2012 to 2014. The dependent variable was the number of Turner syndrome and Down syndrome cases, and the main independent variables were those regarding the water and air pollution. Air pollutants did not have a significant impact on the number of Turner syndrome and Down syndrome cases; however, the increase in number of wastewater discharge companies did have a significant relationship with the number of cases. The more the number of wastewater discharge companies, the more the number Turner syndrome and Down syndrome cases were observed. Therefore, scientific investigation on water and air pollutants in relation with genetic health effects needs to be performed.

Skin symptoms in four ectodermal dysplasia syndromes including two case reports of Rapp-Hodgkin-Syndrome.

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Knaudt, Björn; Volz, Thomas; Krug, Markus; Burgdorf, Walter; Röcken, Martin; Berneburg, Mark

2012-01-01

The skin, hair and nail changes in four distinct ectodermal dysplasia syndromes are compared and reviewed. These syndromes comprise Christ-Siemens-Touraine syndrome; ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome and Rapp-Hodgkin syndrome. A comprehensive overview of the dermatological signs and symptoms in these syndromes was generated from the database of the Ectodermal Dysplasia Network Germany, the clinical findings in the patients seen in our department and an extensive review of the literature. The findings included abnormalities of skin, sweating, hair and nails. These clinical findings are discussed in relation to the underlying molecular defects known to play a role in these four ectodermal dysplasia syndromes.

Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome.

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Roma, Maria; Marden, Colleen L; De Wandele, Inge; Francomano, Clair A; Rowe, Peter C

2018-03-05

To review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research. We searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance. We identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life. There is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings. Copyright © 2018 Elsevier B.V. All rights reserved.

Immature oxidative stress management as a unifying principle in the pathogenesis of necrotizing enterocolitis: insights from an agent-based model.

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Kim, Moses; Christley, Scott; Alverdy, John C; Liu, Donald; An, Gary

2012-02-01

Necrotizing enterocolitis (NEC) is a complex disease involving prematurity, enteral feeding, and bacterial effects. We propose that the underlying initial condition in its pathogenesis is reduced ability of the neonatal gut epithelial cells (NGECs) to clear oxidative stress (OS), and that when such a NGEC population is exposed to enteral feeding, the increased metabolic OS tips the population toward apoptosis, inflammation, bacterial activation, and eventual necrosis. The multi-factorial complexity of NEC requires characterization with computational modeling, and herein, we used an agent-based model (ABM) to instantiate and examine our unifying hypothesis of the pathogenesis of NEC. An ABM of the neonatal gut was created with NGEC computational agents incorporating rules for pathways for OS, p53, tight junctions, Toll-like receptor (TLR)-4, nitric oxide, and nuclear factor-kappa beta (NF-κB). The modeled bacteria activated TLR-4 on contact with NGECs. Simulations included parameter sweeps of OS response, response to feeding, addition of bacteria, and alterations in gut mucus production. The ABM reproduced baseline cellular respiration and clearance of OS. Reduction in OS clearance consistent with clinical NEC led to senescence, apoptosis, or inflammation, with disruption of tight junctions, but rarely to NGEC necrosis. An additional "hit" of bacteria activating TLR-4 potentiated a shift to NGEC necrosis across the entire population. The mucus layer was modeled to limit bacterial-NGEC interactions and reduce this effect, but concomitant apoptosis in the goblet cell population reduced the efficacy of the mucus layer and limited its protective effect in simulated experiments. This finding suggests a means by which increased apoptosis at the cellular population level can lead to a transition to the necrosis outcome. Our ABM incorporates known components of NEC and demonstrates that impaired OS management can lead to apoptosis and inflammation of NGECs, rendering the

Comparative MiRNA Expressional Profiles and Molecular Networks in Human Small Bowel Tissues of Necrotizing Enterocolitis and Spontaneous Intestinal Perforation.

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Pak Cheung Ng

Full Text Available Necrotizing enterocolitis (NEC and spontaneous intestinal perforation (SIP are acute intestinal conditions which could result in mortality and severe morbidity in preterm infants. Our objective was to identify dysregulated micro-RNAs (miRNAs in small bowel tissues of NEC and SIP, and their possible roles in disease pathophysiology.We performed differential miRNA arrays on tissues of NEC (n = 4, SIP (n = 4 and surgical-control (Surg-CTL; n = 4, and validated target miRNAs by qPCR (n = 10 each group. The association of target miRNAs with 52 dysregulated mRNAs was investigated by bioinformatics on functional and base-pair sequence algorithms, and correlation in same tissue samples.We presented the first miRNA profiles of NEC, SIP and Surg-CTL intestinal tissues in preterm infants. Of 28 validated miRNAs, 21 were significantly different between NEC or SIP and Surg-CTL. Limited overlapping in the aberrant expression of miRNAs between NEC and SIP indicated their distinct molecular mechanisms. A proposed network of dysregulated miRNA/mRNA pairs in NEC suggested interaction at bacterial receptor TLR4 (miR-31, miR-451, miR-203, miR-4793-3p, mediated via key transcription factors NFKB2 (miR-203, AP-1/FOSL1 (miR-194-3p, FOXA1 (miR-21-3p, miR-431 and miR-1290 and HIF1A (miR-31, and extended downstream to pathways of angiogenesis, arginine metabolism, cell adhesion and chemotaxis, extracellular matrix remodeling, hypoxia/oxidative stress, inflammation and muscle contraction. In contrast, upregulation of miR-451 and miR-223 in SIP suggested modulation of G-protein-mediated muscle contraction.The robust response of miRNA dysregulation in NEC and SIP, and concerted involvement of specific miRNAs in the molecular networks indicated their crucial roles in mucosa integrity and disease pathophysiology.

Gardner's syndrome

International Nuclear Information System (INIS)

Sobrado Junior, C.W.; Bresser, A.; Cerri, G.G.; Habr-Gama, A.; Pinotti, H.W.; Magalhaes, A.

1988-01-01

A case of familiar poliposis of colon related to a right mandibular osteoma is reported (this association is usually called Gardner's syndrome). Radiologic pictures ae shown and some commentaries about this syndrome concerning the treatment are made. (author) [pt

Confirmation that RIPK4 mutations cause not only Bartsocas-Papas syndrome but also CHAND syndrome.

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Busa, Tiffany; Jeraiby, Mohammed; Clémenson, Alix; Manouvrier, Sylvie; Granados, Viviana; Philip, Nicole; Touraine, Renaud

2017-11-01

CHAND syndrome is an autosomal recessive disorder characterized by curly hair, ankyloblepharon, and nail dysplasia. Only few patients were reported to date. A homozygous RIPK4 mutation was recently identified by homozygosity mapping and whole exome sequencing in three patients from an expanded consanguineous kindred with a clinical diagnosis of CHAND syndrome. RIPK4 was previously known to be implicated in Bartsocas-Papas syndrome, the autosomal recessive form of popliteal pterygium syndrome. We report here two cases of RIPK4 homozygous mutations in a fetus with severe Bartsocas-Papas syndrome and a patient with CHAND syndrome. The patient with CHAND syndrome harbored the same mutation as the one identified in the family previously reported. We thus confirm the implication of RIPK4 gene in CHAND syndrome in addition to Bartsocas-Papas syndrome and discuss genotype/phenotype correlations. © 2017 Wiley Periodicals, Inc.

Multiple strains probiotics appear to be the most effective probiotics in the prevention of necrotizing enterocolitis and mortality: An updated meta-analysis.

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Chang, Hung-Yang; Chen, Jin-Hua; Chang, Jui-Hsing; Lin, Hung-Chih; Lin, Chien-Yu; Peng, Chun-Chih

2017-01-01

Some oral probiotics have been shown to prevent necrotizing enterocolitis (NEC) and decrease mortality effectively in preterm very low birth weight (PVLBW) infants. However, it is unclear whether a single probiotic or a mixture of probiotics is most effective for the prevention of NEC. A meta-analysis was conducted by reviewing the most up to date literature to investigate whether multiple strains probiotics are more effective than a single strain in reducing NEC and death in PVLBW infants. Relevant studies were identified by searches of the MEDLINE, EMBASE, and Cochrane CENTRAL databases, from 2001 to 2016. The inclusion criteria were randomized controlled trials of any enteral probiotic supplementation that was initiated within the first 7 days and continued for at least 14 days in preterm infants (≤ 34 weeks' gestation) and/or those of a birth weight ≤1500 g. A total of 25 trials (n = 7345 infants) were eligible for inclusion in the meta-analysis using a fixed-effects model. Multiple strains probiotics were associated with a marked reduction in the incidence of NEC, with a pooled OR of 0.36 (95% CI, 0.24-0.53; P probiotic using Lactobacillus species had a borderline effect in reducing NEC (OR of 0.60; 95% CI 0.36-1.0; P = .05), but not mortality. Multiple strains probiotics had a greater effectiveness in reducing mortality and were associated with a pooled OR of 0.58 (95% CI, 0.43-0.79; P = .0006). Trials using single strain of Bifidobacterium species and Saccharomyces boulardii did not reveal any beneficial effects in terms of reducing NEC or mortality. This updated report found that multiple strains probiotics appear to be the most feasible and effective strategy for the prevention of NEC and reduction of mortality in PVLBW neonates. Further clinical trials should focus on which probiotic combinations are most effective.

Drug treatment of metabolic syndrome.

Science.gov (United States)

Altabas, Velimir

2013-08-01

The metabolic syndrome is a constellation of risk factors for cardiovascular diseases including: abdominal obesity, a decreased ability to metabolize glucose (increased blood glucose levels and/or presence of insulin resistance), dyslipidemia, and hypertension. Patients who have developed this syndrome have been shown to be at an increased risk of developing cardiovascular disease and/or type 2 diabetes. Genetic factors and the environment both are important in the development of the metabolic syndrome, influencing all single components of this syndrome. The goals of therapy are to treat the underlying cause of the syndrome, to reduce morbidity, and to prevent complications, including premature death. Lifestyle modification is the preferred first-step treatment of the metabolic syndrome. There is no single effective drug treatment affecting all components of the syndrome equally known yet. However, each component of metabolic syndrome has independent goals to be achieved, so miscellaneous types of drugs are used in the treatment of this syndrome, including weight losing drugs, antidiabetics, antihypertensives, antilipemic and anticlothing drugs etc. This article provides a brief insight into contemporary drug treatment of components the metabolic syndrome.

Numerical Magnitude Processing Impairments in Genetic Syndromes: A Cross-Syndrome Comparison of Turner and 22Q11.2 Deletion Syndromes

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Brankaer, Carmen; Ghesquière, Pol; De Wel, Anke; Swillen, Ann; De Smedt, Bert

2017-01-01

Cross-syndrome comparisons offer an important window onto understanding heterogeneity in mathematical learning disabilities or dyscalculia. The present study therefore investigated symbolic numerical magnitude processing in two genetic syndromes that are both characterized by mathematical learning disabilities: Turner syndrome and 22q11.2 deletion…

Neonatal bartter syndrome

International Nuclear Information System (INIS)

Parkash, J.; Salat, S. M.; Khan, I.A.

2006-01-01

A pre-term baby girl was born following a pregnancy complicated by severe polyhydramnios at a gestational age of 36 weeks. She was initially suffering from respiratory distress consistent with idiopathic respiratory distress syndrome, and altered electrolyte imbalance with hyponatremia, hypokalemia and hypochloremic metabolic alkalosis. However, during the third week of life when she had dehydration along with significant electrolyte imbalance, Bartter's syndrome was considered which was supported by findings of high renin and aldosterone levels. Treatment was done by correction of electrolytes and dehydration along with indomethacin. The drug was well tolerated. The infant showed correction of electrolyte imbalance. The features of this case suggest an extreme form of Bartter's syndrome presenting from the early days of life. The syndrome is reported because of it's rarity and alerts pediatricians to the antenatal and neonatal variant of Bartter's syndrome. (author)

Kallmann syndrome and ichthyosis: a case of contiguous gene deletion syndrome

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Irene Berges-Raso

2017-09-01

Full Text Available Kallmann syndrome is a genetically heterogeneous form of hypogonadotropic hypogonadism caused by gonadotropin-releasing hormone deficiency and characterized by anosmia or hyposmia due to hypoplasia of the olfactory bulbs; osteoporosis and metabolic syndrome can develop due to longstanding untreated hypogonadism. Kallmann syndrome affects 1 in 10 000 men and 1 in 50 000 women. Defects in 17 genes, including KAL1, have been implicated. Kallmann syndrome can be associated with X-linked ichthyosis, a skin disorder characterized by early onset dark, dry, irregular scales affecting the limb and trunk, caused by a defect of the steroid sulfatase gene (STS. Both KAL1 and STS are located in the Xp22.3 region; therefore, deletions in this region cause a contiguous gene syndrome. We report the case of a 32-year-old man with ichthyosis referred for evaluation of excessive height (2.07 m and weight (BMI: 29.6 kg/m2, microgenitalia and absence of secondary sex characteristics. We diagnosed Kallmann syndrome with ichthyosis due to a deletion in Xp22.3, a rare phenomenon.

Neonatal respiratory distress syndrome

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Hyaline membrane disease (HMD); Infant respiratory distress syndrome; Respiratory distress syndrome in infants; RDS - infants ... improves slowly after that. Some infants with severe respiratory distress syndrome will die. This most often occurs ...

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

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Prabhas Prasun Giri

2011-01-01

Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

Common input to motor units of intrinsic and extrinsic hand muscles during two-digit object hold.

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Winges, Sara A; Kornatz, Kurt W; Santello, Marco

2008-03-01

Anatomical and physiological evidence suggests that common input to motor neurons of hand muscles is an important neural mechanism for hand control. To gain insight into the synaptic input underlying the coordination of hand muscles, significant effort has been devoted to describing the distribution of common input across motor units of extrinsic muscles. Much less is known, however, about the distribution of common input to motor units belonging to different intrinsic muscles and to intrinsic-extrinsic muscle pairs. To address this void in the literature, we quantified the incidence and strength of near-simultaneous discharges of motor units residing in either the same or different intrinsic hand muscles (m. first dorsal, FDI, and m. first palmar interosseus, FPI) during two-digit object hold. To extend the characterization of common input to pairs of extrinsic muscles (previous work) and pairs of intrinsic muscles (present work), we also recorded electromyographic (EMG) activity from an extrinsic thumb muscle (m. flexor pollicis longus, FPL). Motor-unit synchrony across FDI and FPI was weak (common input strength, CIS, mean +/- SE: 0.17 +/- 0.02). Similarly, motor units from extrinsic-intrinsic muscle pairs were characterized by weak synchrony (FPL-FDI: 0.25 +/- 0.02; FPL-FPI: 0.29 +/- 0.03) although stronger than FDI-FPI. Last, CIS from within FDI and FPI was more than three times stronger (0.70 +/- 0.06 and 0.66 +/- 0.06, respectively) than across these muscles. We discuss present and previous findings within the framework of muscle-pair specific distribution of common input to hand muscles based on their functional role in grasping.

MOMO Syndrome with Holoprosencephaly and Cryptorchidism: Expanding the Spectrum of the New Obesity Syndrome

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Sheetal Sharda

2011-01-01

Full Text Available There are multiple genetic disorders with known or unknown etiology grouped under obesity syndromes. Inspite of having multisystem involvement and often having a characteristic presentation, the understanding of the genetic causes in the majority of these syndromes is still lacking. The common obesity syndromes are Bardet-Biedl, Prader-Willi, Alstrom, Albright's hereditary osteodystrophy, Carpenter, Rubinstein-Taybi, Fragile X, and Börjeson-Forssman-Lehman syndrome. The list is ever increasing as new syndromes are being added to it. One of the recent additions is MOMO syndrome, with about five such cases being reported in literature. Expanding the spectrum of clinical features, we report the first case of MOMO syndrome from India with lobar variant of holoprosencephaly and cryptorchidism, which have not been reported previously.

Duane retraction syndrome type 1 with Usher syndrome type 2: an unreported association.

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Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

2015-05-07

Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. Copyright 2015, SLACK Incorporated.

Rare case of nephrotic syndrome: Schimke syndrome.

Science.gov (United States)

Pedrosa, Anna Kelly Krislane de Vasconcelos; Torres, Luiz Fernando Oliveira; Silva, Ana Corina Brainer Amorim da; Dantas, Adrianna Barros Leal; Zuntini, Káthia Liliane da Cunha Ribeiro; Aguiar, Lia Cordeiro Bastos

2016-01-01

Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case. Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Gorlin-goltz syndrome

International Nuclear Information System (INIS)

Ahmed, N.; Salman, M.; Mansoor, M.A.

2007-01-01

Multiple jaw cysts are a characteristic manifestation of basal cell nevus (Gorlin) syndrome. Gorlin-Goltz syndrome is characterized by symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. This patient showed recurrent jaw and maxillary cysts, for which he was followed for 2 years. (author)

Cardio-renal syndrome

OpenAIRE

Gnanaraj, Joseph; Radhakrishnan, Jai

2016-01-01

Cardio-renal syndrome is a commonly encountered problem in clinical practice. Its pathogenesis is not fully understood. The purpose of this article is to highlight the interaction between the cardiovascular system and the renal system and how their interaction results in the complex syndrome of cardio-renal dysfunction. Additionally, we outline the available therapeutic strategies to manage this complex syndrome.

Tourette Syndrome

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If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are throat- ... spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

Lemierre's syndrome

DEFF Research Database (Denmark)

Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

2014-01-01

Lemierre's syndrome is an often un-diagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation. The syndrome is characterised by infectious thrombosis of the jugular vein due to infection with Fusobacteria, causing...

Sleep overlap syndrome

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Fariba Rezaeetalab

2016-12-01

Full Text Available Overlap syndrome, which is known as the coexistence of chronic obstructive pulmonary disease (COPD and obstructive sleep apnea (OSA, was first defined by Flenley. Although it can refer to concomitant occurrence of any of the pulmonary diseases and OSA, overlap syndrome is commonly considered as the coexistence of OSA and COPD. This disease has unique adverse health consequences distinct from either condition alone. Given the high prevalence of each solitary disease, overlap syndrome is also likely to be common and clinically relevant. Despite the fact that overlap syndrome has been described in the literature for nearly 30 years, paucity of evaluations and studies limited the discussion on diagnosis, prevalence, pathophysiology, treatment, and outcomes of this disease. This review article addresses these issues by reviewing several recent studies conducted in Iran or other countries. This review suggests that overlap syndrome has worse outcomes than either disease alone. Our findings accentuated the urgent need for further studies on overlap syndrome and all overlaps between OSA and chronic pulmonary disease to provide a deeper insight into diagnosis and non-invasive treatments of this disease.

What Causes Cushing's Syndrome?

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... Share Facebook Twitter Pinterest Email Print What causes Cushing syndrome? Cushing syndrome can develop for two reasons: Medication ... uhs ), thyroid, or thymus How Tumors Can Cause Cushing Syndrome Normally, the pituitary gland in the brain controls ...

[The refeeding syndrome].

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Lambers, Wietske M; Kraaijenbrink, Bastiaan; Siegert, Carl E H

2015-01-01

The refeeding syndrome may occur during reintroduction of carbohydrates in malnourished patients. This syndrome is characterized by reduced plasma electrolyte levels, hypophosphataemia being most prevalent. The symptoms can vary from minor symptoms to severe neurological or cardiac symptoms. The pathophysiological mechanism comprises an increase in insulin levels, resulting in shifts of phosphate, potassium and magnesium into the intracellular environment, as well as fluid retention and relative deficiency of vitamin B1. There is growing interest in the screening and treatment of patients with malnutrition, due to which the incidence of refeeding syndrome is probably increasing. Currently, there is no single definition of this syndrome and therefore there is no solid scientific basis for screening and treatment. In this article we describe the rationale for screening and additional laboratory investigations. A prospective, controlled trial is important to define the clinical relevance of the refeeding syndrome and optimize its treatment.

Subclinical nephritic syndrome in children cohabiting with pediatric patients, Presenting acute nephritic syndrome

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Guerrero-Tinoco Gustavo Adolfo; Julio-Barrios Emil

2012-01-01

Introduction: subclinical nephritic syndrome is the presence of hematuria, hypocomplementemiaand/or proteinuria without the presence of signs and/or symptoms.Objective: to determine the incidence of subclinical nephritic syndrome in childrenliving with pediatric patients diagnosed with acute nephritic syndrome.Methods: family visit to identify children living together in the two previous months, with pediatric patients hospitalized with acute nephritic syndrome, at Hospital InfantilNapoleon F...

Relationships among personality traits, metabolic syndrome, and metabolic syndrome scores: The Kakegawa cohort study.

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Ohseto, Hisashi; Ishikuro, Mami; Kikuya, Masahiro; Obara, Taku; Igarashi, Yuko; Takahashi, Satomi; Kikuchi, Daisuke; Shigihara, Michiko; Yamanaka, Chizuru; Miyashita, Masako; Mizuno, Satoshi; Nagai, Masato; Matsubara, Hiroko; Sato, Yuki; Metoki, Hirohito; Tachibana, Hirofumi; Maeda-Yamamoto, Mari; Kuriyama, Shinichi

2018-04-01

Metabolic syndrome and the presence of metabolic syndrome components are risk factors for cardiovascular disease (CVD). However, the association between personality traits and metabolic syndrome remains controversial, and few studies have been conducted in East Asian populations. We measured personality traits using the Japanese version of the Eysenck Personality Questionnaire (Revised Short Form) and five metabolic syndrome components-elevated waist circumference, elevated triglycerides, reduced high-density lipoprotein cholesterol, elevated blood pressure, and elevated fasting glucose-in 1322 participants aged 51.1±12.7years old from Kakegawa city, Japan. Metabolic syndrome score (MS score) was defined as the number of metabolic syndrome components present, and metabolic syndrome as having the MS score of 3 or higher. We performed multiple logistic regression analyses to examine the relationship between personality traits and metabolic syndrome components and multiple regression analyses to examine the relationship between personality traits and MS scores adjusted for age, sex, education, income, smoking status, alcohol use, and family history of CVD and diabetes mellitus. We also examine the relationship between personality traits and metabolic syndrome presence by multiple logistic regression analyses. "Extraversion" scores were higher in those with metabolic syndrome components (elevated waist circumference: P=0.001; elevated triglycerides: P=0.01; elevated blood pressure: P=0.004; elevated fasting glucose: P=0.002). "Extraversion" was associated with the MS score (coefficient=0.12, P=0.0003). No personality trait was significantly associated with the presence of metabolic syndrome. Higher "extraversion" scores were related to higher MS scores, but no personality trait was significantly associated with the presence of metabolic syndrome. Copyright © 2018 Elsevier Inc. All rights reserved.

SNEDDON’S SYNDROME

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Valentin Valtchev

2008-10-01

Full Text Available Sneddon’s syndrome is usually characterized by the association of an ischemic cerebrovascular disease and a widespread livedo reticularis. The incidence of Sneddon syndrome is 4/1000 000. We present 42-year-old woman with livedo reticularis, recurrence ischaemic cerebrovascular accidents, two repetitive miscarriages and positive anti-2GPi antibodies. Skin biopsy specimens reveal inflammatory changes of small- to medium-sized arteries and subendothelial proliferation and fibrosis. The diagnosis Sneddon syndrome is confirmed by skin biopsy, and MR evidence. We suggest that anti-2GPi antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with Sneddon syndrome.

Sjogren′s Syndrome: A Review

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Rani Somani

2011-01-01

Full Text Available Sjogren′s syndrome, also known as "Mikulicz disease" or "Sicca syndrome" is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It can exist by itself (primary Sjogren syndrome or develop in association with another disorder such as rheumatoid arthritis, systemic sclerosis, primary biliary cirrhosis or Hashimoto thyroiditis (associated Sjogren syndrome. Hallmarks are the dry mouth and dry eyes known as the Sicca syndrome. Sjogren syndrome affects t million to 4 million people in the United States- Most are over 40 years old at the time of diagnosis. As there is no known cure for Sjogren syndrome, treatment focuses on relieving symptoms and preventing complications. The most serious complication associated with primary Sjogren syndrome is the development of a lymphoproliferative disease. primarily non-Hodgkin lymphoma.

The ``eco-syndrome`` and what causes it; Das Oeko-Syndrom`` und seine Ausloeser

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Voack, C. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie; Borelli, S. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie; Ring, J. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie

1997-01-24

Headache, tiredness, skin irritation etc. may be the results of adverse effects on health due to toxic substances in residential buildings and at workplaces. Possible causes of syndromes that are hard to objectify but associated with serious afflictions such as the ``eco-syndrome`` or Multiple Chemical Sensitivity (MCS) and the Sick Building Syndrome (SBS) are listed. (VHE) [Deutsch] Bei Kopfschmerzen, Muedigkeit, Hautirritationen u.ae. besteht die Moeglichkeit einer gesundheitlichen Beeintraechtigung durch Schadstoff im Wohn- oder Arbeitsbereich. Moegliche Ausloeser fuer schwer objektivierbare, aber mit hohem Leidensdruck verbundene Krankheitsbilder wie Oeko-Syndrom(Multiple Chemical Sensivity, MCS) und SBS (Sick-Building-Syndrom) werden aufgelistet. (VHE)

[Williams-Beuren syndrome (Williams syndrome). Case report].

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Miklós, Györgyi; Fekete, György; Haltrich, Irén; Tóth, Miklós; Reismann, Péter

2017-11-01

Williams syndrome is a rare genetic disorder, that occurs equally in all ethnic groups and both sexes. The diagnosis might be missed during childhood in mild cases. However, establishing the diagnosis is important, not only to find the cause of intellectual disability but to look for cardiovascular, endocrine, psychiatry, urology and other conditions, which can occur at any age in the patients' lifetime. This case report presents the story of 47-year-old woman, who was admitted with haematemesis. During her stay on the ward, in the light of the distinctive facial features, mental retardation, and social behaviour patterns, the possibility of Williams syndrome emerged. Later, the diagnosis was confirmed by genetic analysis. This female is the oldest living patient with Williams syndrome in Hungary. Orv Hetil. 2017; 158(47): 1883-1888.

The association between the metabolic syndrome and metabolic syndrome score and pulmonary function in non-smoking adults.

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Yoon, Hyun; Gi, Mi Young; Cha, Ju Ae; Yoo, Chan Uk; Park, Sang Muk

2018-03-01

This study assessed the association of metabolic syndrome and metabolic syndrome score with the predicted forced vital capacity and predicted forced expiratory volume in 1 s (predicted forced expiratory volume in 1 s) values in Korean non-smoking adults. We analysed data obtained from 6684 adults during the 2013-2015 Korean National Health and Nutrition Examination Survey. After adjustment for related variables, metabolic syndrome ( p metabolic syndrome score ( p metabolic syndrome score with metabolic syndrome score 0 as a reference group showed no significance for metabolic syndrome score 1 [1.061 (95% confidence interval, 0.755-1.490)] and metabolic syndrome score 2 [1.247 (95% confidence interval, 0.890-1.747)], but showed significant for metabolic syndrome score 3 [1.433 (95% confidence interval, 1.010-2.033)] and metabolic syndrome score ⩾ 4 [1.760 (95% confidence interval, 1.216-2.550)]. In addition, the odds ratio of restrictive pulmonary disease of the metabolic syndrome [1.360 (95% confidence interval, 1.118-1.655)] was significantly higher than those of non-metabolic syndrome. Metabolic syndrome and metabolic syndrome score were inversely associated with the predicted forced vital capacity and forced expiratory volume in 1 s values in Korean non-smoking adults. In addition, metabolic syndrome and metabolic syndrome score were positively associated with the restrictive pulmonary disease.

Cross Syndrome Comparison of Sleep Problems in Children with Down Syndrome and Williams Syndrome

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Ashworth, Anna; Hill, Catherine M.; Karmiloff-Smith, Annette; Dimitriou, Dagmara

2013-01-01

Based on previous findings of frequent sleep problems in children with Down syndrome (DS) and Williams syndrome (WS), the present study aimed to expand our knowledge by using parent report and actigraphy to define sleep problems more precisely in these groups. Twenty-two school-aged children with DS, 24 with WS and 52 typically developing (TD)…

Goldenhar syndrome

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Neeraj Sharma

2013-01-01

Full Text Available Goldenhar syndrome is a syndrome of complex structures developing from first and second branchial arches during blastogenesis. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. The disorder is characterized by a wide spectrum of symptoms and physical features that may vary greatly in range and severity from case to case. Here we present a unique case of Goldenhar syndrome with absence of left condyle, hypoplasia of the zygomatic bone, no pneumatization of the mastoid process, underdeveloped mandible, bifid tongue and the skin tags in the preauricular area.

[Neurobiology of Tourette Syndrome].

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Ünal, Dilek; Akdemir, Devrim

2016-01-01

Tourette Syndrome (TS) is a neurodevelopmental disorder characterized by chronic motor and vocal tics. Although it is a common disorder in childhood, the etiology of Tourette Syndrome has not been fully elucidated yet. Studies, -conducted so far- have revealed differences in neurobiological structures of individuals who suffer from Tourette Syndrome. The objective of this review is to assess etiological and pathophysiological studies in the Tourette Syndrome literature. An electronical search was conducted in PubMed database using the keywords tic disorders, Tourette Syndrome, neurobiology, genetics, neuroimaging and animal models. Research and review studies published between 1985 and 2015, with a selection preference towards recent publications, were reviewed. According to the studies, genetic predisposition hypothesis is considered as a priority. However, a precise genetic disorder associated with Tourette Syndrome has not been found. The evidence from postmortem and neuroimaging studies in heterogenous patient groups and animal studies supports the pathological involvement of cortico-striato-thalamo-cortical (CSTC) circuits in Tourette Syndrome. Consequently, the most emphasized hypothesis in the pathophysiology is the dopaminergic dysfunction in these circuits. Furthermore, these findings of the animal, postmortem and neuroimaging studies have confirmed the neurodevelopmental hypothesis of Tourette Syndrome. In conclusion, more studies are needed to understand the etiology of the disorder. The data obtained from neurobiological studies of the disorder will not only shed light on the way of Tourette Syndrome, but also guide studies on its treatment options.

Genetics Home Reference: Gorlin syndrome

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... for This Condition basal cell nevus syndrome BCNS Gorlin-Goltz syndrome NBCCS nevoid basal cell carcinoma syndrome Related Information ... named? Additional Information & Resources MedlinePlus (2 links) Encyclopedia: Basal Cell Nevus Syndrome Health Topic: Skin Cancer Genetic and Rare Diseases ...

Genetics Home Reference: Rett syndrome

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... Genetic Testing Registry: Rett syndrome Other Diagnosis and Management Resources (4 links) Boston Children's Hospital GeneReview: MECP2-Related Disorders MedlinePlus Encyclopedia: Rett Syndrome RettSyndrome.org: Rett Syndrome Clinics General Information from MedlinePlus (5 links) Diagnostic Tests ...

[Menopause and metabolic syndrome].

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Meirelles, Ricardo M R

2014-03-01

The incidence of cardiovascular disease increases considerably after the menopause. One reason for the increased cardiovascular risk seems to be determined by metabolic syndrome, in which all components (visceral obesity, dyslipidemia, hypertension, and glucose metabolism disorder) are associated with higher incidence of coronary artery disease. After menopause, metabolic syndrome is more prevalent than in premenopausal women, and may plays an important role in the occurrence of myocardial infarction and other atherosclerotic and cardiovascular morbidities. Obesity, an essential component of the metabolic syndrome, is also associated with increased incidence of breast, endometrial, bowel, esophagus, and kidney cancer. The treatment of metabolic syndrome is based on the change in lifestyle and, when necessary, the use of medication directed to its components. In the presence of symptoms of the climacteric syndrome, hormonal therapy, when indicated, will also contribute to the improvement of the metabolic syndrome.

DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome

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Bobot, Mickaël; Coen, Matteo; Simon, Clémentine; Daniel, Laurent; Habib, Gilbert; Serratrice, Jacques

2018-01-01

Abstract Rationale: The life-threatening drug rash with eosinophilia and systemic symptoms (DRESS) syndrome occurs most commonly after exposure to drugs, clinical features mimic those found with other serious systemic disorders. It is rarely associated with thrombotic microangiopathy. Patient concerns: We describe the unique case of a 44-year-old man who simultaneously experienced DRESS syndrome with thrombotic microangiopathy (TMA) after a 5 days treatment with fluindione. Diagnoses: Clinical evaluation leads to the discovery of an underlying lymphangiomatosis, due to a Noonan syndrome. Intervetions: The anticoagulant was withdrawn, and corticosteroids (1 mg/kg/day) and acenocoumarol were started. Outcomes: Clinical improvement ensued. At follow-up the patient is well. Lessons: The association of DRESS with TMA is a rare condition; we believe that the presence of the underlying Noonan syndrome could have been the trigger. Moreover, we speculate about the potential interrelations between these entities. PMID:29642153

A Cross-Syndrome Study of the Development of Holistic Face Recognition in Children with Autism, Down Syndrome, and Williams Syndrome

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Annaz, Dagmara; Karmiloff-Smith, Annette; Johnson, Mark H.; Thomas, Michael S. C.

2009-01-01

We report a cross-syndrome comparison of the development of holistic processing in face recognition in school-aged children with developmental disorders: autism, Down syndrome, and Williams syndrome. The autism group was split into two groups: one with high-functioning children and one with low-functioning children. The latter group has rarely…

Clinical update on metabolic syndrome

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Juan Diego Hernández-Camacho

2017-12-01

Full Text Available Metabolic syndrome has been defined as a global issue since it affects a lot of people. Numerous factors are involved in metabolic syndrome development. It has been described that metabolic syndrome has negative consequences on health. Consequently, a lot of treatments have been proposed to palliate it such as drugs, surgery or life style changes where nutritional habits have shown to be an important point in its management. The current study reviews the literature existing about the actual epidemiology of metabolic syndrome, the components involucrate in its appearance and progression, the clinical consequences of metabolic syndrome and the nutritional strategies reported in its remission. A bibliographic search in PubMed and Medline was performed to identify eligible studies. Authors obtained that metabolic syndrome is present in population from developed and undeveloped areas in a huge scale. Environmental and genetic elements are involucrate in metabolic syndrome development. Metabolic syndrome exponentially increased risk of cardiovascular disease, some types of cancers, diabetes mellitus type 2, sleep disturbances, etc. Nutritional treatments play a crucial role in metabolic syndrome prevention, treatment and recovery.

Genetics Home Reference: Tourette syndrome

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... and Vocal Tic Disorder Gilles de la Tourette Syndrome Gilles de la Tourette's syndrome GTS TD Tourette Disorder Tourette's Disease TS Related ... Additional Information & Resources MedlinePlus (2 links) Encyclopedia: Gilles de la Tourette syndrome Health Topic: Tourette Syndrome Genetic and Rare Diseases ...

Comparison of metabolic syndrome with growing epidemic syndrome Z in terms of risk factors and gender differences.

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Uyar, Meral; Davutoğlu, Vedat; Aydın, Neriman; Filiz, Ayten

2013-05-01

The aim of this study is to compare metabolic syndrome with syndrome Z growing epidemic in terms of risk factors, demographic variables, and gender differences in our large cohort at southeastern area in Turkey. Data of patients admitted to sleep clinic in University of Gaziantep from January 2006 to January 2011 were retrospectively evaluated. ATP III and JNC 7 were used for defining metabolic syndrome and hypertension. Data of 761 patients were evaluated. Hypertension, diabetes mellitus, coronary artery disease, pulmonary hypertension, and left ventricular hypertrophy were more common in patients with syndrome Z than in patients without metabolic syndrome. Age, waist/neck circumferences, BMI, triglyceride, glucose, and Epworth sleepiness scale score were detected higher, whereas the minimum oxygen saturation during sleep was lower in patients with syndrome Z. Metabolic syndrome was more common in sleep apneic subjects than in controls (58 versus 30 %). Female sleep apneics showed higher rate of metabolic syndrome than those of males (74 versus 52 %). Hypertension, diabetes mellitus, coronary artery disease, and left ventricular hypertrophy were detected higher in males with syndrome Z than in males without metabolic syndrome. Snoring and excessive daytime sleepiness were detected higher in females with syndrome Z than in females without metabolic syndrome. Systemic/pulmonary hypertension, diabetes mellitus, and left ventricular hypertrophy were more common in females with syndrome Z than in females without metabolic syndrome. Complaints of headache and systemic/pulmonary hypertension were more common among females than males with syndrome Z. Female syndrome Z patients had lower minimum oxygen saturation than male patients with syndrome Z. Metabolic syndrome in sleep apneic patients is more prevalent than in controls. All metabolic syndrome parameters were significantly different among obstructive sleep apneic patients with respect to gender with more severe

Facts about Down Syndrome

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... monitor children with Down syndrome for these conditions. Treatments Down syndrome is a lifelong condition. Services early in life ... of these services focus on helping children with Down syndrome develop to their ... therapy, and they are typically offered through early intervention ...

LEOPARD-syndrom

DEFF Research Database (Denmark)

Hansen, Lars Kjaersgård; Risby, Kirsten; Bygum, Anette

2009-01-01

We describe a 12-year-old boy with a typical phenotype of the LEOPARD syndrome (LS). The diagnosis was confirmed in the boy and his mother, who both had a mutation in the PTPN11 gene at Thr468Met (c.1403C > T). Several other members of the maternal family are suspected also to have the LEOPARD sy...... syndrome. We discuss the clinical characteristics of LS, the need for follow-up and genetic counselling, and the molecular-genetic background as well as the relationship to the allelic disease Noonan syndrome. Udgivelsesdato: 2009-Jan-26......We describe a 12-year-old boy with a typical phenotype of the LEOPARD syndrome (LS). The diagnosis was confirmed in the boy and his mother, who both had a mutation in the PTPN11 gene at Thr468Met (c.1403C > T). Several other members of the maternal family are suspected also to have the LEOPARD...

[Refeeding syndrome].

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Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it.

Efficacy of syndromic management measured as symptomatic improvement in females with vaginal discharge syndrome.

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Chauhan, Vidyalaxmi; Shah, Maitri C; Patel, Sangita V; Marfatia, Yogesh S; Zalavadiya, Dhara

2016-01-01

In spite of a few shortcomings such as over diagnosis and over treatment, syndromic management is a recommended practice in India for sexually transmitted infections (STIs). This study tries to find out the efficacy of syndromic management measured as symptomatic improvement in females with vaginal discharge syndrome. The objective of the study is to find out the effectiveness of syndromic management in terms of symptomatic improvement among females with vaginal discharge syndrome. A longitudinal study was conducted in Gynecology Department of Tertiary Care Hospital including 180 symptomatic females having vaginal discharge syndrome. Demographic profile, presenting complaints, menstrual history, obstetric history, partner history, and contraceptive history were noted. This was followed by clinical examination and specimen collection for laboratory tests and blood tests to find out type of STI including viral STI such as human immunodeficiency virus (HIV), herpes simplex virus (HSV), and hepatitis B surface antigen (HBsAg). Treatment was given according to syndromic management on the same day. All the participants were asked to come for follow-up after 15 days and their improvement in symptoms was noted as complete improvement, some improvement or no improvement on a five point scale. 63.9% cases showed complete improvement, while 36.1% showed some improvement. None of the patients was without any improvement. Vaginal discharge syndrome was most common between 20 and 30 years (43.4%), and 67.8% of symptomatic females with vaginal discharge syndrome belonged to the lower socioeconomic group. HSV infection was the most common (15%) associated viral infection with vaginal discharge syndrome, while hepatitis B infection was the least common (0.5%). HIV was reactive in 2.8% cases only. Syndromic management was found to be effective in relieving symptoms in most of the cases of vaginal discharge syndrome.

Congenital nephrotic syndrome

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Claudia Fanni

2014-06-01

Full Text Available CNS (Congenital nephrotic syndrome is a disorder characterized by the presence of a nephrotic syndrome in the first three months of life. Different pathologies can cause this syndrome. In general, we can distinguish primary forms (sporadic and hereditary and secondary forms (acquired and associated with other syndromes. The most common form is the Finnish CNS (CNF, congenital nephrotic syndrome of the Finnish type, a hereditary form whose name derives from the fact that the highest incidence is described in that country (1.2:10,000. The pathogenesis, the clinical picture, the diagnostic criteria, the therapy and the outcome are described in details.  Proceedings of the International Course on Perinatal Pathology (part of the 10th International Workshop on Neonatology · October 22nd-25th, 2014 · Cagliari (Italy · October 25th, 2014 · The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken

Late-Onset Nephrotic Syndrome in Galloway-Mowat Syndrome: A Case Report

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Hazza Issa

1999-01-01

Full Text Available Galloway-Mowat Syndrome (GMS has a wide variety of clinical manifestations and histologic findings. All reported cases had developed nephrotic syndrome in the first two years of life. We report a case of 12 years old boy with microcephaly, mental retardation, and typical dysmorphic features of GMS with a late onset of minimal change nephritic syndrome which first manifested at seven years of age.

Prune belly syndrome in an Egyptian infant with Down syndrome: A case report

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Metwalley Kotb A

2008-10-01

Full Text Available Abstract Introduction Prune belly syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. The association between prune belly syndrome and Down syndrome is very rare. Case presentation A 4-month-old Egyptian boy was admitted to our institute for management of acute bronchiolitis. He was born at full term by normal vaginal delivery. His mother, a 42-year-Egyptian villager with six other children, had no antenatal or prenatal care. On examination, the boy was found to be hypotonic. In addition to features of Down syndrome, karyotyping confirmed the diagnosis of trisomy 21. Ultrasound examination of the abdomen showed bilateral gross hydronephrosis with megaureter. Micturating cystourethrography showed grade V vesicoureteric reflux bilaterally with no urethral obstruction. Serum creatinine concentration was 90 μmol/litre, serum sodium was 132 mmol/litre and serum potassium was 5.9 mmol/litre. Conclusion We report an Egyptian infant with Down syndrome and prune belly syndrome. The incidence of this association is unknown. Routine antenatal ultrasonography will help in discovering renal anomalies which can be followed postnatally. Postnatal detection of prune belly syndrome necessitates full radiological investigation to detect any renal anomalies. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.

Prune belly syndrome in an Egyptian infant with Down syndrome: a case report.

Science.gov (United States)

Metwalley, Kotb A; Farghalley, Hekma S; Abd-Elsayed, Alaa A

2008-10-02

Prune belly syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. The association between prune belly syndrome and Down syndrome is very rare. A 4-month-old Egyptian boy was admitted to our institute for management of acute bronchiolitis. He was born at full term by normal vaginal delivery. His mother, a 42-year-Egyptian villager with six other children, had no antenatal or prenatal care. On examination, the boy was found to be hypotonic. In addition to features of Down syndrome, karyotyping confirmed the diagnosis of trisomy 21. Ultrasound examination of the abdomen showed bilateral gross hydronephrosis with megaureter. Micturating cystourethrography showed grade V vesicoureteric reflux bilaterally with no urethral obstruction. Serum creatinine concentration was 90 mumol/litre, serum sodium was 132 mmol/litre and serum potassium was 5.9 mmol/litre. We report an Egyptian infant with Down syndrome and prune belly syndrome. The incidence of this association is unknown. Routine antenatal ultrasonography will help in discovering renal anomalies which can be followed postnatally. Postnatal detection of prune belly syndrome necessitates full radiological investigation to detect any renal anomalies. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.

Simultaneous Occurrence of Duane Retraction Syndrome with Marfan Syndrome

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Mihir Kothari

2011-01-01

Full Text Available Marfan syndrome (MFS is an autosomal dominant disorder of connective tissue, while Duane retraction syndrome (DRS is a congenital cranial dysinnervation disorder (CCDD which can be transmitted as autosomal dominant disorder in 5–10% of patients. In this paper, we present an 8-year-old girl who presented with left eye DRS and bilateral subluxation of the lens associated with MFS in absence of familial involvement. To our knowledge this is the first case report of DRS with MFS. The occurrence of these syndromes together is very rare and appears to be coincidental.

Naumoff short-rib polydactyly syndrome compounded with Mohr oral-facial-digital syndrome

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Young, L.W.; Wilhelm, L.L. [Loma Linda Univ., CA (United States). Medical Center; Zuppan, C.W. [Div. of Pediatric Pathology, Loma Linda University Medical Center, CA (United States); Clark, R. [Div. of Medical Genetics, Loma Linda University Medical Center, CA (United States)

2001-01-01

A stillborn baby boy had findings of severe constitutional dwarfism with short limbs, short ribs, and polydactyly that were consistent with Naumoff (type III) short-rib polydactyly syndrome. He also had additional congenital anomalies, including cleft palate, notching of the upper lip, small tongue with accessory sublingual tissue. These oral and pharyngeal anomalies were consistent with Mohr (type II) oral-facial-digital syndrome. We suggest the stillborn infant represented a compound of Naumoff short-rib polydactyly syndrome (SRPS-III) and Mohr oral-facial-digital syndrome (OFDS-II). (orig.)

The Capgras syndrome in paranoid schizophrenia.

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Silva, J A; Leong, G B

1992-01-01

Capgras syndrome is characterized by a delusion of impostors who are thought to be physically similar but psychologically distinct from the misidentified person. This syndrome is generally thought to be relatively rare. Most of our knowledge about Capgras syndrome derives from single case studies and small series of cases usually from diagnostically heterogeneous groups. In this article, a series of 31 patients suffering from both paranoid schizophrenia and Capgras syndrome is described. Issues pertaining to the phenomenology of Capgras syndrome, the possible relation between Capgras syndrome and other delusional misidentification syndromes, and a neurobiological hypothesis aimed at explaining Capgras syndrome are discussed.

Fahr's Syndrome

Science.gov (United States)

... or 50s, although it can occur at any time in childhood or adolescence. × Definition Fahr's Syndrome is a rare, genetically dominant, inherited ... or 50s, although it can occur at any time in childhood or adolescence. View Full Definition Treatment There is no cure for Fahr's Syndrome, ...

Antiphospholipid syndrome

DEFF Research Database (Denmark)

Cervera, Ricard; Piette, Jean-Charles; Font, Josep

2002-01-01

To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

Role of amino acid supplementation in the prevention of necrotizing enterocolitis in preterm neonates - a review of current evidences.

Science.gov (United States)

Garg, Bhawan Deep; Kabra, Nandkishor S

2018-09-01

Necrotizing enterocolitis (NEC) is one of the most common acute and fatal gastrointestinal emergency in very low birth weight (VLBW) preterm neonates with mortality range from 15 to 30%. NEC is likely due to multifactorial process such as oxidative injury, ischemic necrosis, and over-reactive inflammatory response to intestinal microbes. To evaluate the role of amino acid supplementation for reduction of neonatal NEC in preterm neonates. The literature search was done for various randomized control trial (RCT) by searching the Cochrane Central Register of Controlled Trials (CENTRAL), PubMed, EMBASE, Web of Science, Scopus, Index Copernicus, African Index Medicus (AIM), Thomson Reuters (ESCI), Chemical Abstracts Service (CAS) and other database. This review included 15 RCTs that fulfilled inclusion criteria. The total neonates enrolled in these different RCT are 3424 (amino acid group 1711 and control 1713). Almost all participating neonates were of VLBW or extremely low birth weight (ELBW). In two trials, birth weight was between 1500-2000 grams. The intervention was started within first few days after birth and continued up to 30th day of postnatal age in most of the trials. In two trials, intervention was continued up to 120th day of postnatal age. Arginine, glutamine and N-acetyl cysteine (NAC) were used at the dose of 1.5 mol/kg/day (261 mg/kg/day), 0.3 grams/kg/day and 16-32 mg/kg/day, respectively. Role of amino acid in the prevention of neonatal NEC is not exclusively supported by the current evidence. Only three studies were able to show reduction in the incidence of NEC with amino acid supplementation (arginine, glutamine), and the remaining studies did not report any positive effect. Amino acid supplementation was not associated with significant reduction in mortality due to any causes. However, arginine supplementation was associated with significant reduction in mortality due to NEC. Two studies on glutamine were reported significant reduction in

Teleoperation System with Hybrid Pneumatic-Piezoelectric Actuation for MRI-Guided Needle Insertion with Haptic Feedback

OpenAIRE

Shang, Weijian; Su, Hao; Li, Gang; Fischer, Gregory S.

2013-01-01

This paper presents a surgical master-slave tele-operation system for percutaneous interventional procedures under continuous magnetic resonance imaging (MRI) guidance. This system consists of a piezoelectrically actuated slave robot for needle placement with integrated fiber optic force sensor utilizing Fabry-Perot interferometry (FPI) sensing principle. The sensor flexure is optimized and embedded to the slave robot for measuring needle insertion force. A novel, compact opto-mechanical FPI ...

Eagle's Syndrome

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Pinheiro, Thaís Gonçalves; Soares, Vítor Yamashiro Rocha; Ferreira, Denise Bastos Lage; Raymundo, Igor Teixeira; Nascimento, Luiz Augusto; Oliveira, Carlos Augusto Costa Pires de

2013-01-01

Summary Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT) of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical. PMID:25992033

Eagle's Syndrome

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Pinheiro, Thaís Gonçalves

2014-01-01

Full Text Available Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical.

Review of the refeeding syndrome.

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Kraft, Michael D; Btaiche, Imad F; Sacks, Gordon S

2005-12-01

Refeeding syndrome describes a constellation of metabolic disturbances that occur as a result of reinstitution of nutrition to patients who are starved or severely malnourished. Patients can develop fluid and electrolyte disorders, especially hypophosphatemia, along with neurologic, pulmonary, cardiac, neuromuscular, and hematologic complications. We reviewed literature on refeeding syndrome and the associated electrolyte abnormalities, fluid disturbances, and associated complications. In addition to assessing scientific literature, we also considered clinical experience and judgment in developing recommendations for prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk for developing refeeding syndrome, institute nutrition support cautiously, and correct and supplement electrolyte and vitamin deficiencies to avoid refeeding syndrome. We provide suggestions for the prevention of refeeding syndrome and suggestions for treatment of electrolyte disturbances and complications in patients who develop refeeding syndrome, according to evidence in the literature, the pathophysiology of refeeding syndrome, and clinical experience and judgment.

Heterogeneity in Waardenburg syndrome.

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Hageman, M J; Delleman, J W

1977-01-01

Heterogeneity of Waardenburg syndrome is demonstrated in a review of 1,285 patients from the literature and 34 previously unreported patients in five families in the Netherlands. The syndrome seems to consist of two genetically distinct entities that can be differentiated clinically: type I, Waardenburg syndrome with dystopia canthorum; and type II, Waardenburg syndrome without dystopia canthorum. Both types have an autosomal dominant mode of inheritance. The incidence of bilateral deafness in the two types of the syndrome was found in one-fourth with type I and about half of the patients with type II. This difference has important consequences for genetic counseling. Images Fig. 7 Fig. 8 Fig. 9 PMID:331943

Symptoms and Diagnosis of Metabolic Syndrome

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... Thromboembolism Aortic Aneurysm More Symptoms and Diagnosis of Metabolic Syndrome Updated:Apr 13,2017 What are the symptoms ... Syndrome? This content was last reviewed August 2016. Metabolic Syndrome • Home • About Metabolic Syndrome • Why Metabolic Syndrome Matters • ...

Diagnostik af Dravet syndrom

DEFF Research Database (Denmark)

Hansen, Lars Kjaersgård; Rasmussen, Niels Henrik; Ousager, Lilian Bomme

2010-01-01

Dravet syndrome is an epileptic syndrome of infancy. We describe the features of two cases with genetically verified SCNA1 mutations. The diagnosis was established rather late in one case. The epilepsies were medically intractable and the symptoms characteristic of Dravet syndrome. The children...

[Bilateral "crocodile tears syndrome" associated with Melkersson-Rosenthal syndrome--case report].

Science.gov (United States)

Owecki, Michał K; Kapelusiak-Pielok, Magdalena; Kowal, Piotr; Kozubski, Wojciech

2006-01-01

We present a rare case of bilateral crocodile tears syndrome (CTS) in the course of Melkersson-Rosenthal syndrome. Melkersson-Rosenthal syndrome is characterised by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. The classic triad is infrequent and oligosymptomatic variants are seen more frequently. CTS is a rare complication of facial nerve paralysis characterised by inappropriate lacrimation on the side of the palsy in response to salivary stimuli. It results from aberrant reinnervation of the lacrimal gland by salivary parasympathetic fibres. The therapeutic approach for an acute bout of Melkersson-Rosenthal syndrome consists mainly of steroid administration. CTS management is composed of anticholinergic drugs and surgical procedures. Botulin toxin injection into the lacrimal gland is the most modern therapeutic option. In the case presented CTS developed in a 50-year-old man after 5 incidents of facial palsy due to Melkersson-Rosenthal syndrome. The case deserves attention due to the rarity of the observed symptoms and signs.

Acute nephritic syndrome

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Glomerulonephritis - acute; Acute glomerulonephritis; Nephritis syndrome - acute ... Acute nephritic syndrome is often caused by an immune response triggered by an infection or other disease. Common causes in children ...

The impact of autism spectrum disorder symptoms on gesture use in fragile X syndrome and Down syndrome

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Emily Lorang

2017-12-01

Full Text Available Background & aims This study compared gesture rate and purpose in participants with Down syndrome and fragile X syndrome, and the impact of autism spectrum disorder symptoms on each syndrome. Methods Twenty individuals with fragile X syndrome and 20 individuals with Down syndrome between nine and 22 years of age participated in this study. We coded gesture rate and purpose from an autism spectrum disorder evaluation, the Autism Diagnostic Observation Schedule – Second Edition. Results We did not find between-group differences (Down syndrome compared to fragile X syndrome in gesture rate or purpose. Notably, as autism spectrum disorder symptoms increased, the group with Down syndrome produced a lower rate of gestures, but used gestures for the same purpose. Gesture rate did not change based on autism spectrum disorder symptoms in the participants with fragile X syndrome, but as autism spectrum disorder symptoms increased, the participants with fragile X syndrome produced a larger proportion of gestures to regulate behavior and a smaller proportion for joint attention/social interaction. Conclusions Overall, the amount or purpose of gestures did not differentiate individuals with Down syndrome and fragile X syndrome. However, the presence of autism spectrum disorder symptoms had a significant and unique impact on these genetic disorders. In individuals with Down syndrome, the presence of more autism spectrum disorder symptoms resulted in a reduction in the rate of gesturing, but did not change the purpose. However, in fragile X syndrome, the rate of gestures remained the same, but the purpose of those gestures changed based on autism spectrum disorder symptoms. Implications Autism spectrum disorder symptoms differentially impact gestures in Down syndrome and fragile X syndrome. Individuals with Down syndrome and more autism spectrum disorder symptoms are using gestures less frequently. Therefore, clinicians may need to consider children with

Costello syndrome

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Madhukara J

2007-01-01

Full Text Available Costello syndrome is a rare, distinctive, multiple congenital anomaly syndrome, characterized by soft, loose skin with deep palmar and plantar creases, loose joints, distinctive coarse facial features and skeletal and cardiac abnormalities. The affected patients have a predisposition to develop malignancy, developmental delays and mental retardation. Recently, a 7-year-old male child born to normal nonconsanguineous parents presented to us with abnormal facial features, arrhythmia, mitral valve dysfunction and growth retardation. His cutaneous examination revealed lax and pigmented skin over hands and feet with deep creases, acanthosis nigricans and short curly hairs. Its differentiation from other syndromes with similar clinical features is discussed in this article.

Primary Sjögren’s Syndrome with Sensory Ganglionopathy and Painful Legs and Moving Toes Syndrome

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Mehmet Uğur Çevik

2014-06-01

Full Text Available Sjogren’s syndrome is characterized by the sicca syndrome, with dryness of the mouth (xerostomia and the eyes (xerophthalmia. Sjogren's syndrome is the only connective tissue disease that has been associated with sensory neuronopathy. The syndrome of painful legs and moving toes consisting of pain in the lower limbs with spontaneous movements of the toes or feet. The association between Sjogren’s syndrome and painful legs and moving toes syndrome is a rare condition

Psychosomatic syndromes and anorexia nervosa

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Abbate-Daga Giovanni

2013-01-01

Full Text Available Abstract Background In spite of the role of some psychosomatic factors as alexithymia, mood intolerance, and somatization in both pathogenesis and maintenance of anorexia nervosa (AN, few studies have investigated the prevalence of psychosomatic syndromes in AN. The aim of this study was to use the Diagnostic Criteria for Psychosomatic Research (DCPR to assess psychosomatic syndromes in AN and to evaluate if psychosomatic syndromes could identify subgroups of AN patients. Methods 108 AN inpatients (76 AN restricting subtype, AN-R, and 32 AN binge-purging subtype, AN-BP were consecutively recruited and psychosomatic syndromes were diagnosed with the Structured Interview for DCPR. Participants were asked to complete psychometric tests: Body Shape Questionnaire, Beck Depression Inventory, Eating Disorder Inventory–2, and Temperament and Character Inventory. Data were submitted to cluster analysis. Results Illness denial (63% and alexithymia (54.6% resulted to be the most common syndromes in our sample. Cluster analysis identified three groups: moderate psychosomatic group (49%, somatization group (26%, and severe psychosomatic group (25%. The first group was mainly represented by AN-R patients reporting often only illness denial and alexithymia as DCPR syndromes. The second group showed more severe eating and depressive symptomatology and frequently DCPR syndromes of the somatization cluster. Thanatophobia DCPR syndrome was also represented in this group. The third group reported longer duration of illness and DCPR syndromes were highly represented; in particular, all patients were found to show the alexithymia DCPR syndrome. Conclusions These results highlight the need of a deep assessment of psychosomatic syndromes in AN. Psychosomatic syndromes correlated differently with both severity of eating symptomatology and duration of illness: therefore, DCPR could be effective to achieve tailored treatments.

The Marfan syndrome genetics

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Galina Pungerčič

2005-05-01

Full Text Available Background: The Marfan syndrome is an autosomal dominant heritable disorder of connective tissue. It is caused by mutations in the fibrillin-1 gene encoding glycoprotein fibrillin-1, a component of microfibrils of extracellular matrix. Patients with Marfan syndrome show wide spectra of clinical signs, primarily on skeletal, cardiovascular and ocular organ systems. Cardiovascular complications (especially aortic aneurysm and aortic dissection are the most common cause of mortality of Marfan syndrome patients. Discovering genotype-phenotype correlations is complicated because of the large number of mutations reported as well as clinical heterogeneity among individuals with the same mutation. Despite the progress in the knowledge of the molecular nature of Marfan syndrome the diagnosis is still based mainly on the clinical features in the different body systems.Conclusions: Early identification of patient with Marfan syndrome is of considerable importance because of appropriate treatment that can greatly improve life expectancy. Unfortunately, despite the improvement of diagnostic methods, medical and surgical therapy, the mortality due to undiagnosed Marfan syndrome is still high. The present article reviews the molecular genetic studies of Marfan syndrome since the discovery of the mutations in the fibrillin-1 gene.

A Rare Case of Acute Coronary Syndrome in a Patient With Turner Syndrome.

Science.gov (United States)

Kemaloglu, Tugba; Ozer, Nihat; Fikri Yapici, Mehmet

2016-05-01

In Turner syndrome, cardiovascular complications are the most important causes of early mortality. Congenital cardiovascular abnormalities are found in approximately one third of Turner syndrome patients. Developments in diagnosis and treatment have decreased the rate of mortality related to these abnormalities. In recent years, many papers have mentioned that coronary artery disease developing at early ages in patients with Turner syndrome causes sudden deaths. The patient, a 27-year-old female was admitted to the emergency room with chest pain at rest. She was diagnosed with Turner Syndrome in her teenage years due to amenorrhea. Patients with ECG changes and cardiac enzyme elevations were treated with acute coronary syndrome. The young woman with Turner Syndrome have several risk factors for early Coronary Artery Disease development. In such cases, dramatic results like sudden death or heart attack at an early age may occur in cases of insufficient follow-up and treatment.

Prevalence of dry eye syndrome and Sjogren's syndrome in patients with rheumatoid arthritis.

Science.gov (United States)

Kosrirukvongs, Panida; Ngowyutagon, Panotsom; Pusuwan, Pawana; Koolvisoot, Ajchara; Nilganuwong, Surasak

2012-04-01

Rheumatoid arthritis has manifestations in various organs including ophthalmic involvement. The present study evaluates prevalence of dry eye and secondary Sjogren's syndrome using salivary scintigraphy which has not been used in previous reports. To evaluate the prevalence of secondary Sjogren's syndrome in patients with rheumatoid arthritis, including clinical characteristics and dry eye, compared with non-Sjogren's syndrome. Descriptive cross sectional study Sixty-one patients with rheumatoid arthritis were recruited at Siriraj Hospital during March 2009-September 2010 and filled in the questionnaires about dry eye for Ocular Surface Disease Index (OSDI) with a history taking of associated diseases, medications, duration of symptoms of dry eyes and dry mouth. The Schirmer I test without anesthesia, tear break-up time, rose bengal staining score, severity of keratitis and salivary scintigraphy were measured and analyzed. Prevalence of secondary Sjogren's syndrome and dry eye were 22.2% (95% CI 15.4 to 30.9) and 46.7% (95% CI 38.0 to 55.6), respectively. Dry eye interpreted from OSDI, Schirmer 1 test, tear break-up time and rose bengal staining was 16.4%, 46.7%, 82% and 3.3% respectively. Fifty-two percent of patients had a history of dry eye and dry mouth with mean duration 27.4 and 29.8 months, respectively. Superficial punctate keratitis and abnormal salivary scintigraphy were found in 58.2% and 77.8%. Duration of rheumatoid arthritis, erythrocyte sedimentation rate were not correlated with secondary Sjogren's syndrome. Dry eye from OSDI with secondary Sjogren's syndrome (33.3%) compared with non-Sjogren's syndrome (9.5%) was significant difference (p = 0.008). Adjusted odds ratio for secondary Sjogren's syndrome in OSDIL score > 25 was 13.8 (95% CI 2.6 to 73.8, p = 0.002) compared to OSDI score dry eye syndrome and secondary Sjogren's syndrome in rheumatoid arthritis was crucial for evaluation of their severity and proper management.

Polycystic Ovary Syndrome FAQ

Science.gov (United States)

... Ovary Syndrome (PCOS) • What are common signs and symptoms of polycystic ovary syndrome (PCOS)? • What causes PCOS? • What is insulin resistance? • ... with PCOS? •Glossary What are common signs and symptoms of polycystic ovary syndrome (PCOS)? Common PCOS signs and symptoms include the ...

[Characteristics of traditional Chinese medicine syndromes in patients with acute ischemic stroke of yin or yang syndrome: a multicenter trial].

Science.gov (United States)

You, Jin-song; Huang, Yan; Cai, Ye-feng; Guo, Jian-wen; Liang, Wei-xiong; Huang, Pei-xin; Liu, Mao-cai

2008-04-01

To explore the composition characteristics of traditional Chinese medicine (TCM) syndromes in patients with acute ischemic stroke of yin or yang syndrome by investigating the characteristics of TCM syndromes at different periods after onset. One thousand two hundred and forty-six patients with acute ischemic stroke were admitted in twenty hospitals. According to the "diagnostic criteria of syndrome differentiation of stroke", the characteristics of syndromes in the patients were investigated at the periods of 1-3 days, 4-10 days and 11-30 days after they had ischemic stroke. General distribution of six basic syndromes was compared between the patients with yin syndrome and the patients with yang syndrome at the three periods. The six basic syndromes were wind syndrome, pathogenic fire syndrome, phlegm syndrome, blood stasis syndrome, qi deficiency syndrome, and syndrome of yin deficiency and yang hyperactivity. The percentages of wind, pathogenic fire, and phlegm syndromes in the patients were decreased at the period of 11-30 days as compared with the period of 1-3 days (87.1% vs 79.3%, 52.1% vs 38.7% and 67.1% vs 57.4% respectively, P0.05). There were no differences in the distribution of yin and yang syndromes among the three periods (P>0.05). The percentages of syndromes of wind, pathogenic fire, phlegm, and yin deficiency and yang hyperactivity were higher (Pfour or five syndromes were higher, and the percentages of single-syndromes and complex syndromes of two syndromes were lower in patients with yang syndrome than in patients with yin syndrome (P<0.05, P<0.01). The most frequent complex syndromes in patients with yin syndrome were complex syndrome of wind, phlegm, blood stasis and qi deficiency, and complex syndrome of wind, phlegm and qi deficiency; while the most frequent complex syndromes in patients with yang syndrome were complex syndrome of wind, pathogenic fire, phlegm and qi deficiency, and complex syndrome of wind, pathogenic fire and phlegm. The

Pfeiffer syndrome

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Fryns Jean-Pierre

2006-06-01

Full Text Available Abstract Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

Perinatal features of the RASopathies: Noonan syndrome, cardiofaciocutaneous syndrome and Costello syndrome.

Science.gov (United States)

Myers, Angela; Bernstein, Jonathan A; Brennan, Marie-Luise; Curry, Cynthia; Esplin, Edward D; Fisher, Jamie; Homeyer, Margaret; Manning, Melanie A; Muller, Eric A; Niemi, Anna-Kaisa; Seaver, Laurie H; Hintz, Susan R; Hudgins, Louanne

2014-11-01

The RASopathies are a family of developmental disorders caused by heritable defects of the RAS/MAPK signaling pathway. While the postnatal presentation of this group of disorders is well known, the prenatal and neonatal findings are less widely recognized. We report on the perinatal presentation of 10 patients with Noonan syndrome (NS), nine with Cardiofaciocutaneous syndrome (CFCS) and three with Costello syndrome (CS), in conjunction with the results of a comprehensive literature review. The majority of perinatal findings in NS, CS, and CFCS are shared: polyhydramnios; prematurity; lymphatic dysplasia; macrosomia; relative macrocephaly; respiratory distress; hypotonia, as well as cardiac and renal anomalies. In contrast, fetal arrhythmia and neonatal hypoglycemia are relatively specific to CS. NS, CS, and CFCS should all be considered as a possible diagnosis in pregnancies with a normal karyotype and ultrasound findings of a RASopathy. Recognition of the common perinatal findings of these disorders should facilitate both their prenatal and neonatal diagnosis. © 2014 Wiley Periodicals, Inc.

Mobius syndrome: MRI features

International Nuclear Information System (INIS)

Markarian, Maria F.; Villarroel, Gonzalo M.; Nagel, Jorge R.

2003-01-01

Purpose: Mobius Syndrome or congenital facial diplegia is associated with paralysis of the lateral gaze movements. This syndrome may include other cranial nerve palsies and be associated to musculoskeletal anomalies. Our objective is to show the MRI findings in Mobius Syndrome. Material and methods: MRI study was performed in 3 patients with clinic diagnosis of Mobius Syndrome. RMI (1.5T); exams included axial FSE (T1 and T2), FLAIR, SE/EPI, GRE/20, sagittal FSE T2 , coronal T1, diffusion, angio MRI and Spectroscopy sequences. Results: The common features of this syndrome found in MRI were: depression or straightening of the floor of the fourth ventricle, brainstem anteroposterior diameter diminution, morphologic alteration of the pons and medulla oblongata and of the hypoglossal nuclei as well as severe micrognathia. Conclusion: The morphologic alterations of Mobius Syndrome can be clearly identified by MRI; this method has proved to be a useful diagnostic examination. (author)

Ramsay Hunt syndrome

Science.gov (United States)

Hunt syndrome; Herpes zoster oticus; Geniculate ganglion zoster; Geniculate herpes; Herpetic geniculate ganglionitis ... The varicella-zoster virus that causes Ramsay Hunt syndrome is the same virus that causes chickenpox and ...

[Congenital sensorineural deafness and associated syndromes].

Science.gov (United States)

Moatti, L; Garabedian, E N; Lacombe, H; Spir-Jacob, C

1990-01-01

The etiology of perceptive deafness, especially the congenital variety, requires investigation. The presence of a variety of signs associated with deafness constitutes an "associated syndrome" and helps to define a possible genetic origin. These syndromes only represent a small percentage of overall causes of deafness in children, since at most they account for only 10% of cases. Certain syndromes are encountered more often or are well known, others are extremely rare or have only been described recently. The authors report six of these very rare syndromes discovered among their patients: a KID syndrome, a Leopard syndrome, a Norrie syndrome, a Jervell and Lange Nielsen syndrome, a recently described entity called CEE with deafness and an External Neuro-Cochleo-Pancreatic syndrome which would not appear to have been previously described.

Ambras syndrome

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Sudhir Malwade

2015-01-01

Full Text Available Ambras syndrome, a form of congenital hypertrichosis lanuginosa, is extremely rare in neonates. It is characterized by typical pattern of hair distribution, dysmorphic facial features and a familial pattern of inheritance. We report a case of Ambras syndrome in a preterm neonate with history of consanguinity and positive family history.

Cushing's syndrome during pregnancy

NARCIS (Netherlands)

Mulder, W. J.; Berghout, A.; Wiersinga, W. M.

1990-01-01

Two cases of Cushing's syndrome during pregnancy are reported, both due to an adrenal adenoma. The association of pregnancy and Cushing's syndrome has up to now been described in 48 patients (including our two cases); Cushing's syndrome was ACTH-independent in 59%, ACTH-dependent in 33%, and of

Astrocyte oxidative metabolism and metabolite trafficking after fluid percussion brain injury in adult rats.

Science.gov (United States)

Bartnik-Olson, Brenda L; Oyoyo, Udochukwu; Hovda, David A; Sutton, Richard L

2010-12-01

Despite various lines of evidence pointing to the compartmentation of metabolism within the brain, few studies have reported the effect of a traumatic brain injury (TBI) on neuronal and astrocyte compartments and/or metabolic trafficking between these cells. In this study we used ex vivo ¹³C NMR spectroscopy following an infusion of [1-¹³C] glucose and [1,2-¹³C₂] acetate to study oxidative metabolism in neurons and astrocytes of sham-operated and fluid percussion brain injured (FPI) rats at 1, 5, and 14 days post-surgery. FPI resulted in a decrease in the ¹³C glucose enrichment of glutamate in neurons in the injured hemisphere at day 1. In contrast, enrichment of glutamine in astrocytes from acetate was not significantly decreased at day 1. At day 5 the ¹³C enrichment of glutamate and glutamine from glucose in the injured hemisphere of FPI rats did not differ from sham levels, but glutamine derived from acetate metabolism in astrocytes was significantly increased. The ¹³C glucose enrichment of the C3 position of glutamate (C3) in neurons was significantly decreased ipsilateral to FPI at day 14, whereas the enrichment of glutamine in astrocytes had returned to sham levels at this time point. These findings indicate that the oxidative metabolism of glucose is reduced to a greater extent in neurons compared to astrocytes following a FPI. The increased utilization of acetate to synthesize glutamine, and the acetate enrichment of glutamate via the glutamate-glutamine cycle, suggests an integral protective role for astrocytes in maintaining metabolic function following TBI-induced impairments in glucose metabolism.

A Fabry-Perot interferometer for hard X-rays

International Nuclear Information System (INIS)

Caticha, A.; Caticha-Ellis, S.

1990-01-01

Reflection and transmission coefficients are calculated of a sequence of N thin parallel crystals diffracting close to normal incidence and separated by thin non-diffracting gaps. From the study of the particular case N=2 a new Fabry-Perot interferometer (FPI) is proposed for hard X-rays (wavelengths of the order of Angstroms). It is found that the FPI plates do not need to be carved from a single perfect crystal block but may be grown or cleaved by other methods. This interesting possibility is suggested by two facts. First, it is known that for incidence close to normal various features of dynamical diffraction are much less sensitive to crystal defects and crystal orientation. Second, the existence of a mismatch between the lattices of the two FPI plates may affect but does not qualitatively alter the performance of the FPI. It is shown that the optimal thickness of the plates is of the order of a few microns or less. The plates may be built from a wide variety of materials. The transmission of X-rays is studied as a function of the energy and direction of the incident photons for various values of the plate thickness, of the gap, of the lattice mismatch, and also for different materials in the gap. The transmission profile exhibits a peak which is spectrally very sharp (about 10 -3 eV for low order reflections and even less for higher ones) and which can be easily tuned by changing either the lattice mismatch or the temperature. FPI plates built of materials with low absorption such as graphite seem to be particularly convenient. Several possible applications are suggested. (author)

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome). Case report.

Science.gov (United States)

Fini, G; Belli, E; Mici, E; Virciglio, P; Moricca, L M; D'Itri, L; Leonardi, A; Malavenda, M S; Krizzuk, D; Merola, R; Maturo, A; Pasta, V

2013-01-01

Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome (NBCCS) comprises multiple basal cell carcinomas, keratocysts of the jaw, palmar/plantar pits, spine and rib anomalies, calcifications of the falx cerebri etc. The diagnosis is made according to clinical criteria (Kimonis Criteria) and genetic ones. We studied one family where father and then his sun resulted affected by each syndrome. Gorlin-Goltz syndrome is a rare disease diagnosed according to clinical criteria sometimes difficult to integrate. The family case we presented shows how you can get diagnosis even in older age and after numerous surgeries. Patients should be given special attention and therefore should be monitorized and need multidisciplinary treatments continued in time, even a trivial change of signs and symptoms may be an important indicator of a precipitating event which puts the patient's life under threat.

A rare cause of acute coronary syndrome: Kounis syndrome.

Science.gov (United States)

Almeida, João; Ferreira, Sara; Malheiro, Joana; Fonseca, Paulo; Caeiro, Daniel; Dias, Adelaide; Ribeiro, José; Gama, Vasco

2016-12-01

Kounis syndrome is an acute coronary syndrome in the context of a hypersensitivity reaction. The main pathophysiological mechanism appears to be coronary vasospasm. We report the case of a patient with a history of allergy to quinolones, who was given ciprofloxacin before an elective surgical procedure and during drug administration developed symptoms and electrocardiographic changes suggestive of ST-segment elevation acute coronary syndrome. The drug was suspended and coronary angiography excluded epicardial coronary disease. Two hours after withdrawal of the drug the symptoms and ST elevation had resolved completely. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

Pediatric Toxic Shock Syndrome

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Jennifer Yee

2017-09-01

Full Text Available Audience: This scenario was developed to educate emergency medicine residents on the diagnosis and management of a pediatric patient with toxic shock syndrome. The case is also appropriate for teaching of medical students and advanced practice providers, as well as a review of the principles of crisis resource management, teamwork, and communication. Introduction: Toxic shock syndrome is a low-frequency, high-acuity scenario requiring timely identification and aggressive management. If patients suffering from this condition are managed incorrectly, they may progress into multi-organ dysfunction and potentially death. Toxic shock syndrome has been associated with Streptococcus and Staphylococcus aureus (Staph. Approximately half of Staph cases are associated with menstruation, which was first described in the 1970s-1980s and was associated with the use of absorbent tampons.1 Group A Streptococcus may cause complications such as necrotizing fasciitis and gangrenous myositis.2 Pediatric patients may present critically ill from toxic shock syndrome. Providers need to perform a thorough history and physical exam to discern the source of infection. Management requires aggressive care with antibiotics and IV fluids. Objectives: By the end of this simulation session, the learner will be able to: 1 Recognize toxic shock syndrome. 2 Review the importance of a thorough physical exam. 3 Discuss management of toxic shock syndrome, including supportive care and the difference in antibiotic choices for streptococcal and staphylococcal toxic shock syndrome. 4 Appropriately disposition a patient suffering from toxic shock syndrome. 5 Communicate effectively with team members and nursing staff during a resuscitation of a critically ill patient. Method: This session was conducted using high-fidelity simulation, followed by a debriefing session and lecture on toxic shock syndrome.

Klinefelter's syndrome and Prader-Willi syndrome: A rare combination

NARCIS (Netherlands)

Verhoeven, W.M.A.; Vries, B.B. van; Duffels, S.J.H.; Egger, J.I.M.; Noordam, C.; Tuinier, S.

2007-01-01

In this paper a review is presented of the rare combination of Klinefelter's syndrome and Prader-Willi syndrome (PWS) and a second case of this combination with a uniparental disomy (UPD) etiology of PWS is described. Patients outlined in all other 8 reports and the present case have a PWS

Klinefelter's syndrome and Prader-Willi syndrome: a rare combination.

NARCIS (Netherlands)

Verhoeven, W.M.A.; Vries, B.B. van; Duffels, S.J.H.; Egger, J.I.M.; Noordam, C.; Tuinier, S.

2007-01-01

In this paper a review is presented of the rare combination of Klinefelter's syndrome and Prader-Willi syndrome (PWS) and a second case of this combination with a uniparental disomy (UPD) etiology of PWS is described. Patients outlined in all other 8 reports and the present case have a PWS

Hyperimmunoglobulin E syndrome with Sjogren's syndrome in a child

African Journals Online (AJOL)

We describe a case of hyperimmunoglobulin E syndrome (HIES) associated with Sjogren's syndrome (SS) in a 10-year-old boy and provide a brief review of the literature. Although seen in adults, SS occurring after a primary immunodeficiency disease such as HIES has not previously been reported in a child.

Marfan syndrome (image)

Science.gov (United States)

Marfan syndrome is a disorder of connective tissue which causes skeletal defects typically recognized in a tall, lanky person. A person with Marfan syndrome may exhibit long limbs and spider-like fingers, ...

Hyperimmunoglobulin E syndrome

Science.gov (United States)

... page: //medlineplus.gov/ency/article/001311.htm Hyperimmunoglobulin E syndrome To use the sharing features on this page, please enable JavaScript. Hyperimmunoglobulin E syndrome is a rare, inherited disease. It causes ...

Congenital hypoventilation syndrome and Hirschsprung's disease - Haddad syndrome: A neonatal case presentation.

Science.gov (United States)

Jaiyeola, P; El-Metwally, D; Viscardi, R; Greene, C; Woo, H

2015-01-01

Congenital central hypoventilation syndrome (CCHS) is an uncommon cause of apnea in the newborn characterized by the occurrence of apnea predominantly during sleep. Haddad syndrome is CCHS with Hirschsprung's disease. We report a newborn with Haddad syndrome that had a family history of spinal muscular atrophy and discuss aspects of CCHS and important considerations in the evaluation of apnea in the term newborn.